Neoplasms located in the bone marrow. They are differentiated from neoplasms composed of bone marrow cells, such as MULTIPLE MYELOMA. Most bone marrow neoplasms are metastatic.
Bone marrow diseases, also known as hematologic or blood disorders, refer to conditions that affect the production and function of blood cells within the bone marrow, such as leukemia, lymphoma, myeloma, and aplastic anemia, potentially leading to complications like anemia, neutropenia, thrombocytopenia, and increased susceptibility to infections or bleeding.
The soft tissue filling the cavities of bones. Bone marrow exists in two types, yellow and red. Yellow marrow is found in the large cavities of large bones and consists mostly of fat cells and a few primitive blood cells. Red marrow is a hematopoietic tissue and is the site of production of erythrocytes and granular leukocytes. Bone marrow is made up of a framework of connective tissue containing branching fibers with the frame being filled with marrow cells.
Cells contained in the bone marrow including fat cells (see ADIPOCYTES); STROMAL CELLS; MEGAKARYOCYTES; and the immediate precursors of most blood cells.
A common neoplasm of early childhood arising from neural crest cells in the sympathetic nervous system, and characterized by diverse clinical behavior, ranging from spontaneous remission to rapid metastatic progression and death. This tumor is the most common intraabdominal malignancy of childhood, but it may also arise from thorax, neck, or rarely occur in the central nervous system. Histologic features include uniform round cells with hyperchromatic nuclei arranged in nests and separated by fibrovascular septa. Neuroblastomas may be associated with the opsoclonus-myoclonus syndrome. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, pp2099-2101; Curr Opin Oncol 1998 Jan;10(1):43-51)
The transference of BONE MARROW from one human or animal to another for a variety of purposes including HEMATOPOIETIC STEM CELL TRANSPLANTATION or MESENCHYMAL STEM CELL TRANSPLANTATION.
A specialized CONNECTIVE TISSUE that is the main constituent of the SKELETON. The principle cellular component of bone is comprised of OSTEOBLASTS; OSTEOCYTES; and OSTEOCLASTS, while FIBRILLAR COLLAGENS and hydroxyapatite crystals form the BONE MATRIX.
The continuous turnover of BONE MATRIX and mineral that involves first an increase in BONE RESORPTION (osteoclastic activity) and later, reactive BONE FORMATION (osteoblastic activity). The process of bone remodeling takes place in the adult skeleton at discrete foci. The process ensures the mechanical integrity of the skeleton throughout life and plays an important role in calcium HOMEOSTASIS. An imbalance in the regulation of bone remodeling's two contrasting events, bone resorption and bone formation, results in many of the metabolic bone diseases, such as OSTEOPOROSIS.
Removal of bone marrow and evaluation of its histologic picture.
Progenitor cells from which all blood cells derive.
The amount of mineral per square centimeter of BONE. This is the definition used in clinical practice. Actual bone density would be expressed in grams per milliliter. It is most frequently measured by X-RAY ABSORPTIOMETRY or TOMOGRAPHY, X RAY COMPUTED. Bone density is an important predictor for OSTEOPOROSIS.
Bone loss due to osteoclastic activity.
Techniques for the removal of subpopulations of cells (usually residual tumor cells) from the bone marrow ex vivo before it is infused. The purging is achieved by a variety of agents including pharmacologic agents, biophysical agents (laser photoirradiation or radioisotopes) and immunologic agents. Bone marrow purging is used in both autologous and allogeneic BONE MARROW TRANSPLANTATION.
The development and formation of various types of BLOOD CELLS. Hematopoiesis can take place in the BONE MARROW (medullary) or outside the bone marrow (HEMATOPOIESIS, EXTRAMEDULLARY).
Tumors or cancer located in bone tissue or specific BONES.
Transplantation between individuals of the same species. Usually refers to genetically disparate individuals in contradistinction to isogeneic transplantation for genetically identical individuals.
The growth and development of bones from fetus to adult. It includes two principal mechanisms of bone growth: growth in length of long bones at the epiphyseal cartilages and growth in thickness by depositing new bone (OSTEOGENESIS) with the actions of OSTEOBLASTS and OSTEOCLASTS.
Inbred C57BL mice are a strain of laboratory mice that have been produced by many generations of brother-sister matings, resulting in a high degree of genetic uniformity and homozygosity, making them widely used for biomedical research, including studies on genetics, immunology, cancer, and neuroscience.
Diseases of BONES.
A cytologic technique for measuring the functional capacity of stem cells by assaying their activity.
Progressive restriction of the developmental potential and increasing specialization of function that leads to the formation of specialized cells, tissues, and organs.
Renewal or repair of lost bone tissue. It excludes BONY CALLUS formed after BONE FRACTURES but not yet replaced by hard bone.
Cells propagated in vitro in special media conducive to their growth. Cultured cells are used to study developmental, morphologic, metabolic, physiologic, and genetic processes, among others.
Irradiation of the whole body with ionizing or non-ionizing radiation. It is applicable to humans or animals but not to microorganisms.
Transplantation of an individual's own tissue from one site to another site.
An organism whose body contains cell populations of different genotypes as a result of the TRANSPLANTATION of donor cells after sufficient ionizing radiation to destroy the mature recipient's cells which would otherwise reject the donor cells.
Connective tissue cells of an organ found in the loose connective tissue. These are most often associated with the uterine mucosa and the ovary as well as the hematopoietic system and elsewhere.
The clinical entity characterized by anorexia, diarrhea, loss of hair, leukopenia, thrombocytopenia, growth retardation, and eventual death brought about by the GRAFT VS HOST REACTION.
A form of anemia in which the bone marrow fails to produce adequate numbers of peripheral blood elements.
The process of bone formation. Histogenesis of bone including ossification.
Technique using an instrument system for making, processing, and displaying one or more measurements on individual cells obtained from a cell suspension. Cells are usually stained with one or more fluorescent dyes specific to cell components of interest, e.g., DNA, and fluorescence of each cell is measured as it rapidly transverses the excitation beam (laser or mercury arc lamp). Fluorescence provides a quantitative measure of various biochemical and biophysical properties of the cell, as well as a basis for cell sorting. Other measurable optical parameters include light absorption and light scattering, the latter being applicable to the measurement of cell size, shape, density, granularity, and stain uptake.
A progressive, malignant disease of the blood-forming organs, characterized by distorted proliferation and development of leukocytes and their precursors in the blood and bone marrow. Leukemias were originally termed acute or chronic based on life expectancy but now are classified according to cellular maturity. Acute leukemias consist of predominately immature cells; chronic leukemias are composed of more mature cells. (From The Merck Manual, 2006)
Extracellular substance of bone tissue consisting of COLLAGEN fibers, ground substance, and inorganic crystalline minerals and salts.
The grafting of bone from a donor site to a recipient site.
Glycoproteins found on immature hematopoietic cells and endothelial cells. They are the only molecules to date whose expression within the blood system is restricted to a small number of progenitor cells in the bone marrow.
Leukocytes with abundant granules in the cytoplasm. They are divided into three groups according to the staining properties of the granules: neutrophilic, eosinophilic, and basophilic. Mature granulocytes are the NEUTROPHILS; EOSINOPHILS; and BASOPHILS.
An encapsulated lymphatic organ through which venous blood filters.
Bone-forming cells which secrete an EXTRACELLULAR MATRIX. HYDROXYAPATITE crystals are then deposited into the matrix to form bone.
A large multinuclear cell associated with the BONE RESORPTION. An odontoclast, also called cementoclast, is cytomorphologically the same as an osteoclast and is involved in CEMENTUM resorption.
Transfer of HEMATOPOIETIC STEM CELLS from BONE MARROW or BLOOD between individuals within the same species (TRANSPLANTATION, HOMOLOGOUS) or transfer within the same individual (TRANSPLANTATION, AUTOLOGOUS). Hematopoietic stem cell transplantation has been used as an alternative to BONE MARROW TRANSPLANTATION in the treatment of a variety of neoplasms.
An organism that, as a result of transplantation of donor tissue or cells, consists of two or more cell lines descended from at least two zygotes. This state may result in the induction of donor-specific TRANSPLANTATION TOLERANCE.
A malignancy of mature PLASMA CELLS engaging in monoclonal immunoglobulin production. It is characterized by hyperglobulinemia, excess Bence-Jones proteins (free monoclonal IMMUNOGLOBULIN LIGHT CHAINS) in the urine, skeletal destruction, bone pain, and fractures. Other features include ANEMIA; HYPERCALCEMIA; and RENAL INSUFFICIENCY.
Deficiency of all three cell elements of the blood, erythrocytes, leukocytes and platelets.
Clonal expansion of myeloid blasts in bone marrow, blood, and other tissue. Myeloid leukemias develop from changes in cells that normally produce NEUTROPHILS; BASOPHILS; EOSINOPHILS; and MONOCYTES.
Cell separation is the process of isolating and distinguishing specific cell types or individual cells from a heterogeneous mixture, often through the use of physical or biological techniques.
Elements of limited time intervals, contributing to particular results or situations.
Synthetic or natural materials for the replacement of bones or bone tissue. They include hard tissue replacement polymers, natural coral, hydroxyapatite, beta-tricalcium phosphate, and various other biomaterials. The bone substitutes as inert materials can be incorporated into surrounding tissue or gradually replaced by original tissue.
Metabolic bone diseases are a group of disorders that affect the bones' structure and strength, caused by disturbances in the normal metabolic processes involved in bone formation, resorption, or mineralization, including conditions like osteoporosis, osteomalacia, Paget's disease, and renal osteodystrophy.
Very large BONE MARROW CELLS which release mature BLOOD PLATELETS.
Systemic lysosomal storage disease caused by a deficiency of alpha-L-iduronidase (IDURONIDASE) and characterized by progressive physical deterioration with urinary excretion of DERMATAN SULFATE and HEPARAN SULFATE. There are three recognized phenotypes representing a spectrum of clinical severity from severe to mild: Hurler syndrome, Hurler-Scheie syndrome and Scheie syndrome (formerly mucopolysaccharidosis V). Symptoms may include DWARFISM; hepatosplenomegaly; thick, coarse facial features with low nasal bridge; corneal clouding; cardiac complications; and noisy breathing.
An enzyme that hydrolyzes iduronosidic linkages in desulfated dermatan. Deficiency of this enzyme produces Hurler's syndrome. EC 3.2.1.76.
Mucopolysaccharidosis with excessive CHONDROITIN SULFATE B in urine, characterized by dwarfism and deafness. It is caused by a deficiency of N-ACETYLGALACTOSAMINE-4-SULFATASE (arylsulfatase B).
Mucopolysaccharidosis characterized by excessive dermatan and heparan sulfates in the urine and Hurler-like features. It is caused by a deficiency of beta-glucuronidase.
Mucopolysaccharidosis characterized by heparitin sulfate in the urine, progressive mental retardation, mild dwarfism, and other skeletal disorders. There are four clinically indistinguishable but biochemically distinct forms, each due to a deficiency of a different enzyme.
Therapeutic replacement or supplementation of defective or missing enzymes to alleviate the effects of enzyme deficiency (e.g., GLUCOSYLCERAMIDASE replacement for GAUCHER DISEASE).

Longevity, stress response, and cancer in aging telomerase-deficient mice. (1/551)

Telomere maintenance is thought to play a role in signaling cellular senescence; however, a link with organismal aging processes has not been established. The telomerase null mouse provides an opportunity to understand the effects associated with critical telomere shortening at the organismal level. We studied a variety of physiological processes in an aging cohort of mTR-/- mice. Loss of telomere function did not elicit a full spectrum of classical pathophysiological symptoms of aging. However, age-dependent telomere shortening and accompanying genetic instability were associated with shortened life span as well as a reduced capacity to respond to stresses such as wound healing and hematopoietic ablation. In addition, we found an increased incidence of spontaneous malignancies. These findings demonstrate a critical role for telomere length in the overall fitness, reserve, and well being of the aging organism.  (+info)

Phase I study of 90Y-labeled B72.3 intraperitoneal administration in patients with ovarian cancer: effect of dose and EDTA coadministration on pharmacokinetics and toxicity. (2/551)

The tumor-associated glycoprotein 72 (TAG-72) antigen is present on a high percentage of tumor types including ovarian carcinomas. Antibody B72.3 is a murine monoclonal recognizing the surface domain of the TAG-72 antigen and has been widely used in human clinical trials. After our initial encouraging studies (M. G. Rosenblum et al., J. Natl. Cancer Inst., 83: 1629-1636, 1991) of tissue disposition, metabolism, and pharmacokinetics in 9 patients with ovarian cancer, we designed an escalating dose, multi-arm Phase I study of 90Y-labeled B72.3 i.p. administration. In the first arm of the study, patients (3 pts/dose level) received an i.p. infusion of either 2 or 10 mg of B72.3 labeled with either 1, 10, 15, or 25 mCi of 90Y. Pharmacokinetic studies demonstrated that concentrations of 90Y-labeled B72.3 persist in peritoneal fluid with half-lives >24 h after i.p. administration. In addition, 90Y-labeled B72.3 was absorbed rapidly into the plasma with peak levels achieved within 48 h, and levels declined slowly thereafter. Cumulative urinary excretion of the 90Y label was 10-20% of the administered dose which suggests significant whole-body retention of the radiolabel. Biopsy specimens of bone and marrow obtained at 72 h after administration demonstrated significant content of the label in bone (0.015% of the dose/g) with relatively little in marrow (0.005% of the dose/g). The maximal tolerated dose was determined to be 10 mCi because of hematological toxicity and platelet suppression. This typically occurred on the 29th day after administration and was thought to be a consequence of the irradiation of the marrow from the bony deposition of the radiolabel. In an effort to suppress the bone uptake of 90Y, patients were treated with a continuous i.v. infusion of EDTA (25 mg/kg/12 h x 6) infused immediately before i.p. administration of the radiolabeled antibody. Patients (3 pts/dose level) were treated with doses of 10, 15, 20, 25, 30, 35, 40, or 45 mCi of 90Y-labeled B72.3 for a total of 38 patients. EDTA administration resulted in significant myeloprotection, which allowed escalation to the maximal tolerated dose of 40 mCi. Dose-limiting toxicity was thrombocytopenia and neutropenia. Studies of plasma and peritoneal fluid pharmacokinetics demonstrate no changes compared with patients without EDTA pretreatment. Cumulative urinary excretion of the radiolabel was not increased in patients pretreated with EDTA compared with the untreated group. However, analysis of biopsy specimens of bone and marrow demonstrated that bone and marrow content of the 90Y label was 15-fold lower (<0.001% injected dose/g) than a companion group without EDTA. Four responses were noted in patients who received 15-30 mCi of 90Y-labeled B72.3 with response durations of 1-12 months. These results demonstrate the myeloprotective ability of EDTA, which allows safe i.p. administration of higher doses of 90Y-labeled B72.3 and, therefore, clearly warrant an expanded Phase II trial in patients with minimal residual disease after standard chemotherapy or for the palliation of refractory ascites.  (+info)

Double-blind randomized study on the myeloprotective effect of melatonin in combination with carboplatin and etoposide in advanced lung cancer. (3/551)

A significant myeloprotective effect of melatonin in mice treated with etoposide, cyclophosphamide or carboplatin has been reported. The present study was designed to evaluate if the same effect could be observed in patients receiving chemotherapy. Twenty previously untreated patients with inoperable lung cancer received two cycles of carboplatin (given at area under the curve 5 by the Calvert formula) on day 1 and etoposide (150 mg m(-2) i.v.) on days 1-3 every 4 weeks. Melatonin 40 mg or placebo (double-blind) was given orally in the evening for 21 consecutive days, starting 2 days before chemotherapy. Patients were randomized to receive melatonin either with the first or the second cycle. Complete blood cell count with differential was done three times per week for 3 weeks. The median age of the cohort was 60 years (range 42-69), 16 patients had non-small cell and four patients small-cell lung cancer, 12 stage III and eight stage IV disease. In a multivariate analysis including age, sex, diagnosis, stage, performance status, doses of carboplatin and etoposide, and concomitant treatment with melatonin or placebo, the haematological parameters--depth and duration of toxicity for haemoglobin, platelets and neutrophils (ANC)--were not significantly different between cycles with/without melatonin. The mean ANC nadir and the mean number of days with ANC < 0.5 x 10(9) l(-1) were 0.5 x 10(9) l(-1) and 2.5 days, respectively, with/without melatonin. We concluded that, in patients with lung cancer, melatonin given orally at a dose of 40 mg per day for 21 days in the evening, does not protect against the myelotoxic effect of carboplatin and etoposide.  (+info)

Magnetic resonance imaging of the wrist in early rheumatoid arthritis reveals progression of erosions despite clinical improvement. (4/551)

OBJECTIVES: To investigate the progression of joint damage in early rheumatoid arthritis (RA) using magnetic resonance imaging (MRI) of the wrist and determine whether this technique can be used to predict prognosis. METHODS: An inception cohort of 42 early patients has been followed up prospectively for one year. Gadolinium enhanced MRI scans of the dominant wrist were obtained at baseline and one year and scored for synovitis, tendonitis, bone marrow oedema, and erosions. Plain radiographs were performed concurrently and scored for erosions. Patients were assessed clinically for disease activity and HLA-DRB1 genotyping was performed. RESULTS: At one year, MRI erosions were found in 74% of patients (31 of 42) compared with 45% at baseline. Twelve patients (28.6%) had radiographic erosions at one year. The total MRI score and MRI erosion score increased significantly from baseline to one year despite falls in clinical measures of inflammation including erythrocyte sedimentation rate (ESR), C reactive protein (CRP), and swollen joint count (p < 0.01 for all). Baseline findings that predicted carpal MRI erosions at one year included a total MRI score of 6 or greater (sensitivity: 93.3%, specificity 81.8%, positive predictive value 93.3%, p = 0.000007), MRI bone oedema (OR = 6.47, p < 0.001), MRI synovitis (OR = 2.14, p = 0.003), and pain score (p = 0.01). Radiological erosions at one year were predicted by a total MRI score at baseline of greater than 13 (OR = 12.4, p = 0.002), the presence of MRI erosions (OR = 11.6, p = 0.005), and the ESR (p = 0.02). If MRI erosions were absent at baseline and the total MRI score was low, radiological erosions were highly unlikely to develop by one year (negative predictive value 0.91 and 0.92 respectively). No association was found between the shared epitope and erosions on MRI (p = 0.4) or radiography (p = 1.0) at one year. CONCLUSIONS: MRI scans of the dominant wrist are useful in predicting MRI and radiological erosions in early RA and may indicate the patients that should be managed aggressively. Discordance has been demonstrated between clinical improvement and progression of MRI erosion scores.  (+info)

Hematopoietic damage prior to PBSCT and its influence on hematopoietic recovery. (5/551)

BACKGROUND AND OBJECTIVE: Patients with malignancies receive chemotherapy to induce tumor remission which could damage hematopoiesis and adversely influence hematopoietic reconstitution after transplantation. In the present study we used a long-term culture (LTBMC) system and clonogenic assays to evaluate the marrow damage in patients selected to receive peripheral blood stem cell transplantation (PBSCT). DESIGN AND METHODS: Thirty-five patients - 20 with breast cancer (BC), 9 with non-Hodgkin's lymphoma (NHL) and 6 with Hodgkin's disease (HD) - were included. Bone marrow aspiration was performed one day prior to the initiation of the conditioning therapy. CFU-GM were cultured in methylcellulose with PHA-LCM. Delta assays of plastic adherent progenitor cells (PD) were performed according to Gordon's method. LTBMC were established for 5 weeks. RESULTS: There were fewer CFU-GM from all patient groups than from normal BM (p<0.05). In contrast, the number of immature progenitor cells (PD) was not decreased. The total number of CFU-GM produced by LTBMC patients was significantly reduced (p<0.05). The adherent layer from patients was often qualitatively different. In order to know whether the hematopoietic damage could affect hematopoietic reconstitution, we correlated culture data with time taken to reach peripheral cell counts. A negative correlation (r= - 0.71) was found between percentage of stromal layer and time taken to reach 20x10(9) platelets/L (tplat= 20x3-0.08% stromal layer). INTERPRETATION AND CONCLUSIONS: We can conclude that prior to PBSCT, hematopoietic function is impaired at both the level of committed progenitor cells and that of BM stroma. This damage could influence platelet recovery.  (+info)

First-line high-dose sequential chemotherapy with rG-CSF and repeated blood stem cell transplantation in untreated inflammatory breast cancer: toxicity and response (PEGASE 02 trial). (6/551)

Despite the generalization of induction chemotherapy and a better outcome for chemosensitive diseases, the prognosis of inflammatory breast cancer (IBC) is still poor. In this work, we evaluate response and toxicity of high-dose sequential chemotherapy with repeated blood stem cell (BSC) transplantation administered as initial treatment in 100 women with non-metastatic IBC. Ninety-five patients (five patients were evaluated as non-eligible) of median age 46 years (range 26-56) received four cycles of chemotherapy associating: cyclophosphamide (C) 6 g m(-2) - doxorubicin (D) 75 mg m(-2) cycle 1, C: 3 g m(-2) - D: 75 mg m(-2) cycle 2, C: 3 g m(-2) - D: 75 mg m(-2) - 5 FU 2500 mg m(-2) cycle 3 and 4. BSC were collected after cycle 1 or 2 and reinfused after cycle 3 and 4. rG-CSF was administered after the four cycles. Mastectomy and radiotherapy were planned after chemotherapy completion. Pathological response was considered as the first end point of this trial. A total of 366 cycles of chemotherapy were administered. Eighty-seven patients completed the four cycles and relative dose intensity was respectively 0.97 (range 0.4-1.04) and 0.96 (range 0.25-1.05) for C and D. Main toxicity was haematological with febrile neutropenia ranging from 26% to 51% of cycles; one death occurred during aplasia. Clinical response rate was 90% +/- 6%. Eighty-six patients underwent mastectomy in a median of 3.5 months (range 3-9) after the first cycle of chemotherapy; pathological complete response rate in breast was 32% +/- 10%. All patients were eligible to receive additional radiotherapy. High-dose chemotherapy with repeated BSC transplantation is feasible with acceptable toxicity in IBC. Pathological response rate is encouraging but has to be confirmed by final outcome.  (+info)

Ticlopidine and clopidogrel. (7/551)

The thienopyridines ticlopidine and clopidogrel are inhibitors of platelet function in vivo. Their mode of action has not been defined, but it appears that they require conversion to as yet unidentified metabolites that are noncompetitive antagonists of the platelet ADP receptor. Inhibition of platelet aggregation with these compounds is delayed until 24 to 48 hours after administration. Maximum inhibition occurs after 3 to 5 days, and recovery is slow after drug withdrawal. Ticlopidine is effective in preventing cardiovascular events in cerebrovascular, cardiovascular, and peripheral vascular disease, with an efficacy that is similar to aspirin. However, its use is associated with significant and sometimes fatal adverse reactions, specifically neutropenia and bone marrow aplasia. Gastrointestinal side effects and skin rashes are common and result in discontinuation of therapy in up to 10% of patients. Clopidogrel is at least as effective as aspirin in preventing cardiovascular events in patients with a history of vascular disease. It appears to be safer than ticlopidine, although its efficacy in acute coronary syndromes or post-coronary-stent insertion has not been reported. Important outstanding issues are whether clopidogrel adds to the benefit of aspirin and whether the combination of these agents is safe. If so, this combination may become the standard for antithrombotic therapy in cardiovascular disease.  (+info)

Measurement of vitamin B12-binding proteins of plasma. II. Interpretation of patterns in disease. (8/551)

The technique described in the preceding paper was applied to 12 abnormal sera selected for their increase in one or more B12-binding proteins. Even in the presence of large amounts of R-type binder, the ammonium sulfate technique gave a reliable separation of R binding proteins from TC II. Measurement of the TC II in abnormal sera gave results identical to those obtained by the more standard gel filtration. The R binders of four subjects with myeloproliferative disease were further separated into alpha2-R and alpha1-R. The pattern of B12 binding of polycythemia vera (PV) was an exaggeration of the normal pattern. Binding to alpha2-R was three to four times that to alpha1-R, although the total amounts bound to both were increased. In chronic myelogenous leukemia (CML), both alpha2-R and alpha1-R were also increased, but in contrast to binding in normal sera, alpha1-R predominated. In order to interpret the findings, either whole serum R or alpha1-R and alpha2-R from patients with myeloproliferative disease were subject to isoelectric focusing. Alpha2-R consisted pricipally of components isoelectric at pH 2.9, 3.0, and 3.1. These components were present in only minor amounts in normal serum and were somewhat increased in the serum of PV. These components were very much increased in the serum of CML and predominated. Alpha2-R consisted of those components isoelectric at pH 3.4,3.6, and 4.0. These components predominated in the unsaturated binding capacity of normal sera and that of PV. It was concluded that the division of plasma R binders into alpha1-R and alpha1-R by the technique described provided information useful in the study of myeloproliferative diseases.  (+info)

Bone marrow neoplasms are a type of cancer that originates in the bone marrow, which is the spongy tissue inside bones where blood cells are produced. These neoplasms can be divided into two main categories: hematologic (or liquid) malignancies and solid tumors.

Hematologic malignancies include leukemias, lymphomas, and multiple myeloma. Leukemias are cancers of the white blood cells, which normally fight infections. In leukemia, the bone marrow produces abnormal white blood cells that do not function properly, leading to an increased risk of infection, anemia, and bleeding.

Lymphomas are cancers of the lymphatic system, which helps to fight infections and remove waste from the body. Lymphoma can affect the lymph nodes, spleen, thymus gland, and bone marrow. There are two main types of lymphoma: Hodgkin's lymphoma and non-Hodgkin's lymphoma.

Multiple myeloma is a cancer of the plasma cells, which are a type of white blood cell that produces antibodies to help fight infections. In multiple myeloma, abnormal plasma cells accumulate in the bone marrow and produce large amounts of abnormal antibodies, leading to bone damage, anemia, and an increased risk of infection.

Solid tumors of the bone marrow are rare and include conditions such as chordomas, Ewing sarcomas, and osteosarcomas. These cancers originate in the bones themselves or in other tissues that support the bones, but they can also spread to the bone marrow.

Treatment for bone marrow neoplasms depends on the type and stage of cancer, as well as the patient's overall health. Treatment options may include chemotherapy, radiation therapy, stem cell transplantation, targeted therapy, or a combination of these approaches.

Bone marrow diseases, also known as hematologic disorders, are conditions that affect the production and function of blood cells in the bone marrow. The bone marrow is the spongy tissue inside bones where all blood cells are produced. There are various types of bone marrow diseases, including:

1. Leukemia: A cancer of the blood-forming tissues, including the bone marrow. Leukemia causes the body to produce large numbers of abnormal white blood cells, which can crowd out healthy blood cells and impair their function.
2. Lymphoma: A cancer that starts in the lymphatic system, which is part of the immune system. Lymphoma can affect the bone marrow and cause an overproduction of abnormal white blood cells.
3. Multiple myeloma: A cancer of the plasma cells, a type of white blood cell found in the bone marrow. Multiple myeloma causes an overproduction of abnormal plasma cells, which can lead to bone pain, fractures, and other complications.
4. Aplastic anemia: A condition in which the bone marrow does not produce enough new blood cells. This can lead to symptoms such as fatigue, weakness, and an increased risk of infection.
5. Myelodysplastic syndromes (MDS): A group of disorders in which the bone marrow does not produce enough healthy blood cells. MDS can lead to anemia, infections, and bleeding.
6. Myeloproliferative neoplasms (MPNs): A group of disorders in which the bone marrow produces too many abnormal white or red blood cells, or platelets. MPNs can lead to symptoms such as fatigue, itching, and an increased risk of blood clots.

Treatment for bone marrow diseases depends on the specific condition and its severity. Treatment options may include chemotherapy, radiation therapy, stem cell transplantation, or targeted therapies that target specific genetic mutations.

Bone marrow is the spongy tissue found inside certain bones in the body, such as the hips, thighs, and vertebrae. It is responsible for producing blood-forming cells, including red blood cells, white blood cells, and platelets. There are two types of bone marrow: red marrow, which is involved in blood cell production, and yellow marrow, which contains fatty tissue.

Red bone marrow contains hematopoietic stem cells, which can differentiate into various types of blood cells. These stem cells continuously divide and mature to produce new blood cells that are released into the circulation. Red blood cells carry oxygen throughout the body, white blood cells help fight infections, and platelets play a crucial role in blood clotting.

Bone marrow also serves as a site for immune cell development and maturation. It contains various types of immune cells, such as lymphocytes, macrophages, and dendritic cells, which help protect the body against infections and diseases.

Abnormalities in bone marrow function can lead to several medical conditions, including anemia, leukopenia, thrombocytopenia, and various types of cancer, such as leukemia and multiple myeloma. Bone marrow aspiration and biopsy are common diagnostic procedures used to evaluate bone marrow health and function.

Bone marrow cells are the types of cells found within the bone marrow, which is the spongy tissue inside certain bones in the body. The main function of bone marrow is to produce blood cells. There are two types of bone marrow: red and yellow. Red bone marrow is where most blood cell production takes place, while yellow bone marrow serves as a fat storage site.

The three main types of bone marrow cells are:

1. Hematopoietic stem cells (HSCs): These are immature cells that can differentiate into any type of blood cell, including red blood cells, white blood cells, and platelets. They have the ability to self-renew, meaning they can divide and create more hematopoietic stem cells.
2. Red blood cell progenitors: These are immature cells that will develop into mature red blood cells, also known as erythrocytes. Red blood cells carry oxygen from the lungs to the body's tissues and carbon dioxide back to the lungs.
3. Myeloid and lymphoid white blood cell progenitors: These are immature cells that will develop into various types of white blood cells, which play a crucial role in the body's immune system by fighting infections and diseases. Myeloid progenitors give rise to granulocytes (neutrophils, eosinophils, and basophils), monocytes, and megakaryocytes (which eventually become platelets). Lymphoid progenitors differentiate into B cells, T cells, and natural killer (NK) cells.

Bone marrow cells are essential for maintaining a healthy blood cell count and immune system function. Abnormalities in bone marrow cells can lead to various medical conditions, such as anemia, leukopenia, leukocytosis, thrombocytopenia, or thrombocytosis, depending on the specific type of blood cell affected. Additionally, bone marrow cells are often used in transplantation procedures to treat patients with certain types of cancer, such as leukemia and lymphoma, or other hematologic disorders.

Neuroblastoma is defined as a type of cancer that develops from immature nerve cells found in the fetal or early postnatal period, called neuroblasts. It typically occurs in infants and young children, with around 90% of cases diagnosed before age five. The tumors often originate in the adrenal glands but can also arise in the neck, chest, abdomen, or spine. Neuroblastoma is characterized by its ability to spread (metastasize) to other parts of the body, including bones, bone marrow, lymph nodes, and skin. The severity and prognosis of neuroblastoma can vary widely, depending on factors such as the patient's age at diagnosis, stage of the disease, and specific genetic features of the tumor.

Bone marrow transplantation (BMT) is a medical procedure in which damaged or destroyed bone marrow is replaced with healthy bone marrow from a donor. Bone marrow is the spongy tissue inside bones that produces blood cells. The main types of BMT are autologous, allogeneic, and umbilical cord blood transplantation.

In autologous BMT, the patient's own bone marrow is used for the transplant. This type of BMT is often used in patients with lymphoma or multiple myeloma who have undergone high-dose chemotherapy or radiation therapy to destroy their cancerous bone marrow.

In allogeneic BMT, bone marrow from a genetically matched donor is used for the transplant. This type of BMT is often used in patients with leukemia, lymphoma, or other blood disorders who have failed other treatments.

Umbilical cord blood transplantation involves using stem cells from umbilical cord blood as a source of healthy bone marrow. This type of BMT is often used in children and adults who do not have a matched donor for allogeneic BMT.

The process of BMT typically involves several steps, including harvesting the bone marrow or stem cells from the donor, conditioning the patient's body to receive the new bone marrow or stem cells, transplanting the new bone marrow or stem cells into the patient's body, and monitoring the patient for signs of engraftment and complications.

BMT is a complex and potentially risky procedure that requires careful planning, preparation, and follow-up care. However, it can be a life-saving treatment for many patients with blood disorders or cancer.

"Bone" is the hard, dense connective tissue that makes up the skeleton of vertebrate animals. It provides support and protection for the body's internal organs, and serves as a attachment site for muscles, tendons, and ligaments. Bone is composed of cells called osteoblasts and osteoclasts, which are responsible for bone formation and resorption, respectively, and an extracellular matrix made up of collagen fibers and mineral crystals.

Bones can be classified into two main types: compact bone and spongy bone. Compact bone is dense and hard, and makes up the outer layer of all bones and the shafts of long bones. Spongy bone is less dense and contains large spaces, and makes up the ends of long bones and the interior of flat and irregular bones.

The human body has 206 bones in total. They can be further classified into five categories based on their shape: long bones, short bones, flat bones, irregular bones, and sesamoid bones.

Bone remodeling is the normal and continuous process by which bone tissue is removed from the skeleton (a process called resorption) and new bone tissue is formed (a process called formation). This ongoing cycle allows bones to repair microdamage, adjust their size and shape in response to mechanical stress, and maintain mineral homeostasis. The cells responsible for bone resorption are osteoclasts, while the cells responsible for bone formation are osteoblasts. These two cell types work together to maintain the structural integrity and health of bones throughout an individual's life.

During bone remodeling, the process can be divided into several stages:

1. Activation: The initiation of bone remodeling is triggered by various factors such as microdamage, hormonal changes, or mechanical stress. This leads to the recruitment and activation of osteoclast precursor cells.
2. Resorption: Osteoclasts attach to the bone surface and create a sealed compartment called a resorption lacuna. They then secrete acid and enzymes that dissolve and digest the mineralized matrix, creating pits or cavities on the bone surface. This process helps remove old or damaged bone tissue and releases calcium and phosphate ions into the bloodstream.
3. Reversal: After resorption is complete, the osteoclasts undergo apoptosis (programmed cell death), and mononuclear cells called reversal cells appear on the resorbed surface. These cells prepare the bone surface for the next stage by cleaning up debris and releasing signals that attract osteoblast precursors.
4. Formation: Osteoblasts, derived from mesenchymal stem cells, migrate to the resorbed surface and begin producing a new organic matrix called osteoid. As the osteoid mineralizes, it forms a hard, calcified structure that gradually replaces the resorbed bone tissue. The osteoblasts may become embedded within this newly formed bone as they differentiate into osteocytes, which are mature bone cells responsible for maintaining bone homeostasis and responding to mechanical stress.
5. Mineralization: Over time, the newly formed bone continues to mineralize, becoming stronger and more dense. This process helps maintain the structural integrity of the skeleton and ensures adequate calcium storage.

Throughout this continuous cycle of bone remodeling, hormones, growth factors, and mechanical stress play crucial roles in regulating the balance between resorption and formation. Disruptions to this delicate equilibrium can lead to various bone diseases, such as osteoporosis, where excessive resorption results in weakened bones and increased fracture risk.

A bone marrow examination is a medical procedure in which a sample of bone marrow, the spongy tissue inside bones where blood cells are produced, is removed and examined. This test is used to diagnose or monitor various conditions affecting blood cell production, such as infections, leukemia, anemia, and other disorders of the bone marrow.

The sample is typically taken from the hipbone (iliac crest) or breastbone (sternum) using a special needle. The procedure may be done under local anesthesia or with sedation to minimize discomfort. Once the sample is obtained, it is examined under a microscope for the presence of abnormal cells, changes in cell size and shape, and other characteristics that can help diagnose specific conditions. Various stains, cultures, and other tests may also be performed on the sample to provide additional information.

Bone marrow examination is an important diagnostic tool in hematology and oncology, as it allows for a detailed assessment of blood cell production and can help guide treatment decisions for patients with various blood disorders.

Hematopoietic stem cells (HSCs) are immature, self-renewing cells that give rise to all the mature blood and immune cells in the body. They are capable of both producing more hematopoietic stem cells (self-renewal) and differentiating into early progenitor cells that eventually develop into red blood cells, white blood cells, and platelets. HSCs are found in the bone marrow, umbilical cord blood, and peripheral blood. They have the ability to repair damaged tissues and offer significant therapeutic potential for treating various diseases, including hematological disorders, genetic diseases, and cancer.

Bone density refers to the amount of bone mineral content (usually measured in grams) in a given volume of bone (usually measured in cubic centimeters). It is often used as an indicator of bone strength and fracture risk. Bone density is typically measured using dual-energy X-ray absorptiometry (DXA) scans, which provide a T-score that compares the patient's bone density to that of a young adult reference population. A T-score of -1 or above is considered normal, while a T-score between -1 and -2.5 indicates osteopenia (low bone mass), and a T-score below -2.5 indicates osteoporosis (porous bones). Regular exercise, adequate calcium and vitamin D intake, and medication (if necessary) can help maintain or improve bone density and prevent fractures.

Bone resorption is the process by which bone tissue is broken down and absorbed into the body. It is a normal part of bone remodeling, in which old or damaged bone tissue is removed and new tissue is formed. However, excessive bone resorption can lead to conditions such as osteoporosis, in which bones become weak and fragile due to a loss of density. This process is carried out by cells called osteoclasts, which break down the bone tissue and release minerals such as calcium into the bloodstream.

Bone marrow purging is a procedure that involves the removal of cancerous or damaged cells from bone marrow before it is transplanted into a patient. This process is often used in the treatment of blood cancers such as leukemia and lymphoma, as well as other diseases that affect the bone marrow.

The purging process typically involves collecting bone marrow from the patient or a donor, then treating it with chemicals or medications to eliminate any cancerous or damaged cells. The purged bone marrow is then transplanted back into the patient's body, where it can help to produce healthy new blood cells.

There are several methods that can be used for bone marrow purging, including physical separation techniques, chemical treatments, and immunological approaches using antibodies or other immune system components. The choice of method depends on several factors, including the type and stage of the disease being treated, as well as the patient's individual medical history and condition.

It is important to note that bone marrow purging is a complex procedure that carries some risks and potential complications, such as damage to healthy cells, delayed recovery, and increased risk of infection. As with any medical treatment, it should be carefully evaluated and discussed with a healthcare provider to determine whether it is appropriate for a given patient's situation.

Hematopoiesis is the process of forming and developing blood cells. It occurs in the bone marrow and includes the production of red blood cells (erythropoiesis), white blood cells (leukopoiesis), and platelets (thrombopoiesis). This process is regulated by various growth factors, hormones, and cytokines. Hematopoiesis begins early in fetal development and continues throughout a person's life. Disorders of hematopoiesis can result in conditions such as anemia, leukopenia, leukocytosis, thrombocytopenia, or thrombocytosis.

Bone neoplasms are abnormal growths or tumors that develop in the bone. They can be benign (non-cancerous) or malignant (cancerous). Benign bone neoplasms do not spread to other parts of the body and are rarely a threat to life, although they may cause problems if they grow large enough to press on surrounding tissues or cause fractures. Malignant bone neoplasms, on the other hand, can invade and destroy nearby tissue and may spread (metastasize) to other parts of the body.

There are many different types of bone neoplasms, including:

1. Osteochondroma - a benign tumor that develops from cartilage and bone
2. Enchondroma - a benign tumor that forms in the cartilage that lines the inside of the bones
3. Chondrosarcoma - a malignant tumor that develops from cartilage
4. Osteosarcoma - a malignant tumor that develops from bone cells
5. Ewing sarcoma - a malignant tumor that develops in the bones or soft tissues around the bones
6. Giant cell tumor of bone - a benign or occasionally malignant tumor that develops from bone tissue
7. Fibrosarcoma - a malignant tumor that develops from fibrous tissue in the bone

The symptoms of bone neoplasms vary depending on the type, size, and location of the tumor. They may include pain, swelling, stiffness, fractures, or limited mobility. Treatment options depend on the type and stage of the tumor but may include surgery, radiation therapy, chemotherapy, or a combination of these treatments.

Homologous transplantation is a type of transplant surgery where organs or tissues are transferred between two genetically non-identical individuals of the same species. The term "homologous" refers to the similarity in structure and function of the donated organ or tissue to the recipient's own organ or tissue.

For example, a heart transplant from one human to another is an example of homologous transplantation because both organs are hearts and perform the same function. Similarly, a liver transplant, kidney transplant, lung transplant, and other types of organ transplants between individuals of the same species are also considered homologous transplantations.

Homologous transplantation is in contrast to heterologous or xenogeneic transplantation, where organs or tissues are transferred from one species to another, such as a pig heart transplanted into a human. Homologous transplantation is more commonly performed than heterologous transplantation due to the increased risk of rejection and other complications associated with xenogeneic transplants.

Bone development, also known as ossification, is the process by which bone tissue is formed and grows. This complex process involves several different types of cells, including osteoblasts, which produce new bone matrix, and osteoclasts, which break down and resorb existing bone tissue.

There are two main types of bone development: intramembranous and endochondral ossification. Intramembranous ossification occurs when bone tissue forms directly from connective tissue, while endochondral ossification involves the formation of a cartilage model that is later replaced by bone.

During fetal development, most bones develop through endochondral ossification, starting as a cartilage template that is gradually replaced by bone tissue. However, some bones, such as those in the skull and clavicles, develop through intramembranous ossification.

Bone development continues after birth, with new bone tissue being laid down and existing tissue being remodeled throughout life. This ongoing process helps to maintain the strength and integrity of the skeleton, allowing it to adapt to changing mechanical forces and repair any damage that may occur.

C57BL/6 (C57 Black 6) is an inbred strain of laboratory mouse that is widely used in biomedical research. The term "inbred" refers to a strain of animals where matings have been carried out between siblings or other closely related individuals for many generations, resulting in a population that is highly homozygous at most genetic loci.

The C57BL/6 strain was established in 1920 by crossing a female mouse from the dilute brown (DBA) strain with a male mouse from the black strain. The resulting offspring were then interbred for many generations to create the inbred C57BL/6 strain.

C57BL/6 mice are known for their robust health, longevity, and ease of handling, making them a popular choice for researchers. They have been used in a wide range of biomedical research areas, including studies of cancer, immunology, neuroscience, cardiovascular disease, and metabolism.

One of the most notable features of the C57BL/6 strain is its sensitivity to certain genetic modifications, such as the introduction of mutations that lead to obesity or impaired glucose tolerance. This has made it a valuable tool for studying the genetic basis of complex diseases and traits.

Overall, the C57BL/6 inbred mouse strain is an important model organism in biomedical research, providing a valuable resource for understanding the genetic and molecular mechanisms underlying human health and disease.

Bone diseases is a broad term that refers to various medical conditions that affect the bones. These conditions can be categorized into several groups, including:

1. Developmental and congenital bone diseases: These are conditions that affect bone growth and development before or at birth. Examples include osteogenesis imperfecta (brittle bone disease), achondroplasia (dwarfism), and cleidocranial dysostosis.
2. Metabolic bone diseases: These are conditions that affect the body's ability to maintain healthy bones. They are often caused by hormonal imbalances, vitamin deficiencies, or problems with mineral metabolism. Examples include osteoporosis, osteomalacia, and Paget's disease of bone.
3. Inflammatory bone diseases: These are conditions that cause inflammation in the bones. They can be caused by infections, autoimmune disorders, or other medical conditions. Examples include osteomyelitis, rheumatoid arthritis, and ankylosing spondylitis.
4. Degenerative bone diseases: These are conditions that cause the bones to break down over time. They can be caused by aging, injury, or disease. Examples include osteoarthritis, avascular necrosis, and diffuse idiopathic skeletal hyperostosis (DISH).
5. Tumors and cancers of the bone: These are conditions that involve abnormal growths in the bones. They can be benign or malignant. Examples include osteosarcoma, chondrosarcoma, and Ewing sarcoma.
6. Fractures and injuries: While not strictly a "disease," fractures and injuries are common conditions that affect the bones. They can result from trauma, overuse, or weakened bones. Examples include stress fractures, compound fractures, and dislocations.

Overall, bone diseases can cause a wide range of symptoms, including pain, stiffness, deformity, and decreased mobility. Treatment for these conditions varies depending on the specific diagnosis but may include medication, surgery, physical therapy, or lifestyle changes.

A Colony-Forming Units (CFU) assay is a type of laboratory test used to measure the number of viable, or living, cells in a sample. It is commonly used to enumerate bacteria, yeast, and other microorganisms. The test involves placing a known volume of the sample onto a nutrient-agar plate, which provides a solid growth surface for the cells. The plate is then incubated under conditions that allow the cells to grow and form colonies. Each colony that forms on the plate represents a single viable cell from the original sample. By counting the number of colonies and multiplying by the known volume of the sample, the total number of viable cells in the sample can be calculated. This information is useful in a variety of applications, including monitoring microbial populations, assessing the effectiveness of disinfection procedures, and studying microbial growth and survival.

Cell differentiation is the process by which a less specialized cell, or stem cell, becomes a more specialized cell type with specific functions and structures. This process involves changes in gene expression, which are regulated by various intracellular signaling pathways and transcription factors. Differentiation results in the development of distinct cell types that make up tissues and organs in multicellular organisms. It is a crucial aspect of embryonic development, tissue repair, and maintenance of homeostasis in the body.

Bone regeneration is the biological process of new bone formation that occurs after an injury or removal of a portion of bone. This complex process involves several stages, including inflammation, migration and proliferation of cells, matrix deposition, and mineralization, leading to the restoration of the bone's structure and function.

The main cells involved in bone regeneration are osteoblasts, which produce new bone matrix, and osteoclasts, which resorb damaged or old bone tissue. The process is tightly regulated by various growth factors, hormones, and signaling molecules that promote the recruitment, differentiation, and activity of these cells.

Bone regeneration can occur naturally in response to injury or surgical intervention, such as fracture repair or dental implant placement. However, in some cases, bone regeneration may be impaired due to factors such as age, disease, or trauma, leading to delayed healing or non-union of the bone. In these situations, various strategies and techniques, including the use of bone grafts, scaffolds, and growth factors, can be employed to enhance and support the bone regeneration process.

"Cells, cultured" is a medical term that refers to cells that have been removed from an organism and grown in controlled laboratory conditions outside of the body. This process is called cell culture and it allows scientists to study cells in a more controlled and accessible environment than they would have inside the body. Cultured cells can be derived from a variety of sources, including tissues, organs, or fluids from humans, animals, or cell lines that have been previously established in the laboratory.

Cell culture involves several steps, including isolation of the cells from the tissue, purification and characterization of the cells, and maintenance of the cells in appropriate growth conditions. The cells are typically grown in specialized media that contain nutrients, growth factors, and other components necessary for their survival and proliferation. Cultured cells can be used for a variety of purposes, including basic research, drug development and testing, and production of biological products such as vaccines and gene therapies.

It is important to note that cultured cells may behave differently than they do in the body, and results obtained from cell culture studies may not always translate directly to human physiology or disease. Therefore, it is essential to validate findings from cell culture experiments using additional models and ultimately in clinical trials involving human subjects.

Whole-Body Irradiation (WBI) is a medical procedure that involves the exposure of the entire body to a controlled dose of ionizing radiation, typically used in the context of radiation therapy for cancer treatment. The purpose of WBI is to destroy cancer cells or suppress the immune system prior to a bone marrow transplant. It can be delivered using various sources of radiation, such as X-rays, gamma rays, or electrons, and is carefully planned and monitored to minimize harm to healthy tissues while maximizing the therapeutic effect on cancer cells. Potential side effects include nausea, vomiting, fatigue, and an increased risk of infection due to decreased white blood cell counts.

Autologous transplantation is a medical procedure where cells, tissues, or organs are removed from a person, stored and then returned back to the same individual at a later time. This is different from allogeneic transplantation where the tissue or organ is obtained from another donor. The term "autologous" is derived from the Greek words "auto" meaning self and "logos" meaning study.

In autologous transplantation, the patient's own cells or tissues are used to replace or repair damaged or diseased ones. This reduces the risk of rejection and eliminates the need for immunosuppressive drugs, which are required in allogeneic transplants to prevent the body from attacking the foreign tissue.

Examples of autologous transplantation include:

* Autologous bone marrow or stem cell transplantation, where stem cells are removed from the patient's blood or bone marrow, stored and then reinfused back into the same individual after high-dose chemotherapy or radiation therapy to treat cancer.
* Autologous skin grafting, where a piece of skin is taken from one part of the body and transplanted to another area on the same person.
* Autologous chondrocyte implantation, where cartilage cells are harvested from the patient's own knee, cultured in a laboratory and then implanted back into the knee to repair damaged cartilage.

A radiation chimera is not a widely used or recognized medical term. However, in the field of genetics and radiation biology, a "chimera" refers to an individual that contains cells with different genetic backgrounds. A radiation chimera, therefore, could refer to an organism that has become a chimera as a result of exposure to radiation, which can cause mutations and changes in the genetic makeup of cells.

Ionizing radiation, such as that used in cancer treatments or nuclear accidents, can cause DNA damage and mutations in cells. If an organism is exposed to radiation and some of its cells undergo mutations while others do not, this could result in a chimera with genetically distinct populations of cells.

However, it's important to note that the term "radiation chimera" is not commonly used in medical literature or clinical settings. If you encounter this term in a different context, I would recommend seeking clarification from the source to ensure a proper understanding.

Stromal cells, also known as stromal/stroma cells, are a type of cell found in various tissues and organs throughout the body. They are often referred to as the "connective tissue" or "supporting framework" of an organ because they play a crucial role in maintaining the structure and function of the tissue. Stromal cells include fibroblasts, adipocytes (fat cells), and various types of progenitor/stem cells. They produce and maintain the extracellular matrix, which is the non-cellular component of tissues that provides structural support and biochemical cues for other cells. Stromal cells also interact with immune cells and participate in the regulation of the immune response. In some contexts, "stromal cells" can also refer to cells found in the microenvironment of tumors, which can influence cancer growth and progression.

Graft-versus-host disease (GVHD) is a condition that can occur after an allogeneic hematopoietic stem cell transplantation (HSCT), where the donated immune cells (graft) recognize the recipient's tissues (host) as foreign and attack them. This results in inflammation and damage to various organs, particularly the skin, gastrointestinal tract, and liver.

Acute GVHD typically occurs within 100 days of transplantation and is characterized by symptoms such as rash, diarrhea, and liver dysfunction. Chronic GVHD, on the other hand, can occur after 100 days or even years post-transplant and may present with a wider range of symptoms, including dry eyes and mouth, skin changes, lung involvement, and issues with mobility and flexibility in joints.

GVHD is a significant complication following allogeneic HSCT and can have a substantial impact on the patient's quality of life and overall prognosis. Preventative measures, such as immunosuppressive therapy, are often taken to reduce the risk of GVHD, but its management remains a challenge in transplant medicine.

Aplastic anemia is a medical condition characterized by pancytopenia (a decrease in all three types of blood cells: red blood cells, white blood cells, and platelets) due to the failure of bone marrow to produce new cells. It is called "aplastic" because the bone marrow becomes hypocellular or "aplastic," meaning it contains few or no blood-forming stem cells.

The condition can be acquired or inherited, with acquired aplastic anemia being more common. Acquired aplastic anemia can result from exposure to toxic chemicals, radiation, drugs, viral infections, or autoimmune disorders. Inherited forms of the disease include Fanconi anemia and dyskeratosis congenita.

Symptoms of aplastic anemia may include fatigue, weakness, shortness of breath, pale skin, easy bruising or bleeding, frequent infections, and fever. Treatment options for aplastic anemia depend on the severity of the condition and its underlying cause. They may include blood transfusions, immunosuppressive therapy, and stem cell transplantation.

Osteogenesis is the process of bone formation or development. It involves the differentiation and maturation of osteoblasts, which are bone-forming cells that synthesize and deposit the organic matrix of bone tissue, composed mainly of type I collagen. This organic matrix later mineralizes to form the inorganic crystalline component of bone, primarily hydroxyapatite.

There are two primary types of osteogenesis: intramembranous and endochondral. Intramembranous osteogenesis occurs directly within connective tissue, where mesenchymal stem cells differentiate into osteoblasts and form bone tissue without an intervening cartilage template. This process is responsible for the formation of flat bones like the skull and clavicles.

Endochondral osteogenesis, on the other hand, involves the initial development of a cartilaginous model or template, which is later replaced by bone tissue. This process forms long bones, such as those in the limbs, and occurs through several stages involving chondrocyte proliferation, hypertrophy, and calcification, followed by invasion of blood vessels and osteoblasts to replace the cartilage with bone tissue.

Abnormalities in osteogenesis can lead to various skeletal disorders and diseases, such as osteogenesis imperfecta (brittle bone disease), achondroplasia (a form of dwarfism), and cleidocranial dysplasia (a disorder affecting skull and collarbone development).

Flow cytometry is a medical and research technique used to measure physical and chemical characteristics of cells or particles, one cell at a time, as they flow in a fluid stream through a beam of light. The properties measured include:

* Cell size (light scatter)
* Cell internal complexity (granularity, also light scatter)
* Presence or absence of specific proteins or other molecules on the cell surface or inside the cell (using fluorescent antibodies or other fluorescent probes)

The technique is widely used in cell counting, cell sorting, protein engineering, biomarker discovery and monitoring disease progression, particularly in hematology, immunology, and cancer research.

Leukemia is a type of cancer that originates from the bone marrow - the soft, inner part of certain bones where new blood cells are made. It is characterized by an abnormal production of white blood cells, known as leukocytes or blasts. These abnormal cells accumulate in the bone marrow and interfere with the production of normal blood cells, leading to a decrease in red blood cells (anemia), platelets (thrombocytopenia), and healthy white blood cells (leukopenia).

There are several types of leukemia, classified based on the specific type of white blood cell affected and the speed at which the disease progresses:

1. Acute Leukemias - These types of leukemia progress rapidly, with symptoms developing over a few weeks or months. They involve the rapid growth and accumulation of immature, nonfunctional white blood cells (blasts) in the bone marrow and peripheral blood. The two main categories are:
- Acute Lymphoblastic Leukemia (ALL) - Originates from lymphoid progenitor cells, primarily affecting children but can also occur in adults.
- Acute Myeloid Leukemia (AML) - Develops from myeloid progenitor cells and is more common in older adults.

2. Chronic Leukemias - These types of leukemia progress slowly, with symptoms developing over a period of months to years. They involve the production of relatively mature, but still abnormal, white blood cells that can accumulate in large numbers in the bone marrow and peripheral blood. The two main categories are:
- Chronic Lymphocytic Leukemia (CLL) - Affects B-lymphocytes and is more common in older adults.
- Chronic Myeloid Leukemia (CML) - Originates from myeloid progenitor cells, characterized by the presence of a specific genetic abnormality called the Philadelphia chromosome. It can occur at any age but is more common in middle-aged and older adults.

Treatment options for leukemia depend on the type, stage, and individual patient factors. Treatments may include chemotherapy, targeted therapy, immunotherapy, stem cell transplantation, or a combination of these approaches.

Bone matrix refers to the non-cellular component of bone that provides structural support and functions as a reservoir for minerals, such as calcium and phosphate. It is made up of organic and inorganic components. The organic component consists mainly of type I collagen fibers, which provide flexibility and tensile strength to the bone. The inorganic component is primarily composed of hydroxyapatite crystals, which give bone its hardness and compressive strength. Bone matrix also contains other proteins, growth factors, and signaling molecules that regulate bone formation, remodeling, and repair.

Bone transplantation, also known as bone grafting, is a surgical procedure in which bone or bone-like material is transferred from one part of the body to another or from one person to another. The graft may be composed of cortical (hard outer portion) bone, cancellous (spongy inner portion) bone, or a combination of both. It can be taken from different sites in the same individual (autograft), from another individual of the same species (allograft), or from an animal source (xenograft). The purpose of bone transplantation is to replace missing bone, provide structural support, and stimulate new bone growth. This procedure is commonly used in orthopedic, dental, and maxillofacial surgeries to repair bone defects caused by trauma, tumors, or congenital conditions.

CD34 is a type of antigen that is found on the surface of certain cells in the human body. Specifically, CD34 antigens are present on hematopoietic stem cells, which are immature cells that can develop into different types of blood cells. These stem cells are found in the bone marrow and are responsible for producing red blood cells, white blood cells, and platelets.

CD34 antigens are a type of cell surface marker that is used in medical research and clinical settings to identify and isolate hematopoietic stem cells. They are also used in the development of stem cell therapies and transplantation procedures. CD34 antigens can be detected using various laboratory techniques, such as flow cytometry or immunohistochemistry.

It's important to note that while CD34 is a useful marker for identifying hematopoietic stem cells, it is not exclusive to these cells and can also be found on other cell types, such as endothelial cells that line blood vessels. Therefore, additional markers are often used in combination with CD34 to more specifically identify and isolate hematopoietic stem cells.

Granulocytes are a type of white blood cell that plays a crucial role in the body's immune system. They are called granulocytes because they contain small granules in their cytoplasm, which are filled with various enzymes and proteins that help them fight off infections and destroy foreign substances.

There are three types of granulocytes: neutrophils, eosinophils, and basophils. Neutrophils are the most abundant type and are primarily responsible for fighting bacterial infections. Eosinophils play a role in defending against parasitic infections and regulating immune responses. Basophils are involved in inflammatory reactions and allergic responses.

Granulocytes are produced in the bone marrow and released into the bloodstream, where they circulate and patrol for any signs of infection or foreign substances. When they encounter a threat, they quickly move to the site of infection or injury and release their granules to destroy the invading organisms or substances.

Abnormal levels of granulocytes in the blood can indicate an underlying medical condition, such as an infection, inflammation, or a bone marrow disorder.

The spleen is an organ in the upper left side of the abdomen, next to the stomach and behind the ribs. It plays multiple supporting roles in the body:

1. It fights infection by acting as a filter for the blood. Old red blood cells are recycled in the spleen, and platelets and white blood cells are stored there.
2. The spleen also helps to control the amount of blood in the body by removing excess red blood cells and storing platelets.
3. It has an important role in immune function, producing antibodies and removing microorganisms and damaged red blood cells from the bloodstream.

The spleen can be removed without causing any significant problems, as other organs take over its functions. This is known as a splenectomy and may be necessary if the spleen is damaged or diseased.

Osteoblasts are specialized bone-forming cells that are derived from mesenchymal stem cells. They play a crucial role in the process of bone formation and remodeling. Osteoblasts synthesize, secrete, and mineralize the organic matrix of bones, which is mainly composed of type I collagen.

These cells have receptors for various hormones and growth factors that regulate their activity, such as parathyroid hormone, vitamin D, and transforming growth factor-beta. When osteoblasts are not actively producing bone matrix, they can become trapped within the matrix they produce, where they differentiate into osteocytes, which are mature bone cells that play a role in maintaining bone structure and responding to mechanical stress.

Abnormalities in osteoblast function can lead to various bone diseases, such as osteoporosis, osteogenesis imperfecta, and Paget's disease of bone.

Osteoclasts are large, multinucleated cells that are primarily responsible for bone resorption, a process in which they break down and dissolve the mineralized matrix of bones. They are derived from monocyte-macrophage precursor cells of hematopoietic origin and play a crucial role in maintaining bone homeostasis by balancing bone formation and bone resorption.

Osteoclasts adhere to the bone surface and create an isolated microenvironment, called the "resorption lacuna," between their cell membrane and the bone surface. Here, they release hydrogen ions into the lacuna through a process called proton pumping, which lowers the pH and dissolves the mineral component of the bone matrix. Additionally, osteoclasts secrete proteolytic enzymes, such as cathepsin K, that degrade the organic components, like collagen, in the bone matrix.

An imbalance in osteoclast activity can lead to various bone diseases, including osteoporosis and Paget's disease, where excessive bone resorption results in weakened and fragile bones.

Hematopoietic Stem Cell Transplantation (HSCT) is a medical procedure where hematopoietic stem cells (immature cells that give rise to all blood cell types) are transplanted into a patient. This procedure is often used to treat various malignant and non-malignant disorders affecting the hematopoietic system, such as leukemias, lymphomas, multiple myeloma, aplastic anemia, inherited immune deficiency diseases, and certain genetic metabolic disorders.

The transplantation can be autologous (using the patient's own stem cells), allogeneic (using stem cells from a genetically matched donor, usually a sibling or unrelated volunteer), or syngeneic (using stem cells from an identical twin).

The process involves collecting hematopoietic stem cells, most commonly from the peripheral blood or bone marrow. The collected cells are then infused into the patient after the recipient's own hematopoietic system has been ablated (or destroyed) using high-dose chemotherapy and/or radiation therapy. This allows the donor's stem cells to engraft, reconstitute, and restore the patient's hematopoietic system.

HSCT is a complex and potentially risky procedure with various complications, including graft-versus-host disease, infections, and organ damage. However, it offers the potential for cure or long-term remission in many patients with otherwise fatal diseases.

A transplantation chimera is a rare medical condition that occurs after an organ or tissue transplant, where the recipient's body accepts and integrates the donor's cells or tissues to such an extent that the two sets of DNA coexist and function together. This phenomenon can lead to the presence of two different genetic profiles in one individual.

In some cases, this may result in the development of donor-derived cells or organs within the recipient's body, which can express the donor's unique genetic traits. Transplantation chimerism is more commonly observed in bone marrow transplants, where the donor's immune cells can repopulate and establish themselves within the recipient's bone marrow and bloodstream.

It is important to note that while transplantation chimerism can be beneficial for the success of the transplant, it may also pose some risks, such as an increased likelihood of developing graft-versus-host disease (GVHD), where the donor's immune cells attack the recipient's tissues.

Multiple myeloma is a type of cancer that forms in a type of white blood cell called a plasma cell. Plasma cells help your body fight infection by producing antibodies. In multiple myeloma, cancerous plasma cells accumulate in the bone marrow and crowd out healthy blood cells. Rather than producing useful antibodies, the cancer cells produce abnormal proteins that can cause complications such as kidney damage, bone pain and fractures.

Multiple myeloma is a type of cancer called a plasma cell neoplasm. Plasma cell neoplasms are diseases in which there is an overproduction of a single clone of plasma cells. In multiple myeloma, this results in the crowding out of normal plasma cells, red and white blood cells and platelets, leading to many of the complications associated with the disease.

The abnormal proteins produced by the cancer cells can also cause damage to organs and tissues in the body. These abnormal proteins can be detected in the blood or urine and are often used to monitor the progression of multiple myeloma.

Multiple myeloma is a relatively uncommon cancer, but it is the second most common blood cancer after non-Hodgkin lymphoma. It typically occurs in people over the age of 65, and men are more likely to develop multiple myeloma than women. While there is no cure for multiple myeloma, treatments such as chemotherapy, radiation therapy, and stem cell transplantation can help manage the disease and its symptoms, and improve quality of life.

Pancytopenia is a medical condition characterized by a reduction in the number of all three types of blood cells in the peripheral blood: red blood cells (anemia), white blood cells (leukopenia), and platelets (thrombocytopenia). This condition can be caused by various underlying diseases, including bone marrow disorders, viral infections, exposure to toxic substances or radiation, vitamin deficiencies, and certain medications. Symptoms of pancytopenia may include fatigue, weakness, increased susceptibility to infections, and easy bruising or bleeding.

Acute myeloid leukemia (AML) is a type of cancer that originates in the bone marrow, the soft inner part of certain bones where new blood cells are made. In AML, the immature cells, called blasts, in the bone marrow fail to mature into normal blood cells. Instead, these blasts accumulate and interfere with the production of normal blood cells, leading to a shortage of red blood cells (anemia), platelets (thrombocytopenia), and normal white blood cells (leukopenia).

AML is called "acute" because it can progress quickly and become severe within days or weeks without treatment. It is a type of myeloid leukemia, which means that it affects the myeloid cells in the bone marrow. Myeloid cells are a type of white blood cell that includes monocytes and granulocytes, which help fight infection and defend the body against foreign invaders.

In AML, the blasts can build up in the bone marrow and spread to other parts of the body, including the blood, lymph nodes, liver, spleen, and brain. This can cause a variety of symptoms, such as fatigue, fever, frequent infections, easy bruising or bleeding, and weight loss.

AML is typically treated with a combination of chemotherapy, radiation therapy, and/or stem cell transplantation. The specific treatment plan will depend on several factors, including the patient's age, overall health, and the type and stage of the leukemia.

Cell separation is a process used to separate and isolate specific cell types from a heterogeneous mixture of cells. This can be accomplished through various physical or biological methods, depending on the characteristics of the cells of interest. Some common techniques for cell separation include:

1. Density gradient centrifugation: In this method, a sample containing a mixture of cells is layered onto a density gradient medium and then centrifuged. The cells are separated based on their size, density, and sedimentation rate, with denser cells settling closer to the bottom of the tube and less dense cells remaining near the top.

2. Magnetic-activated cell sorting (MACS): This technique uses magnetic beads coated with antibodies that bind to specific cell surface markers. The labeled cells are then passed through a column placed in a magnetic field, which retains the magnetically labeled cells while allowing unlabeled cells to flow through.

3. Fluorescence-activated cell sorting (FACS): In this method, cells are stained with fluorochrome-conjugated antibodies that recognize specific cell surface or intracellular markers. The stained cells are then passed through a laser beam, which excites the fluorophores and allows for the detection and sorting of individual cells based on their fluorescence profile.

4. Filtration: This simple method relies on the physical size differences between cells to separate them. Cells can be passed through filters with pore sizes that allow smaller cells to pass through while retaining larger cells.

5. Enzymatic digestion: In some cases, cells can be separated by enzymatically dissociating tissues into single-cell suspensions and then using various separation techniques to isolate specific cell types.

These methods are widely used in research and clinical settings for applications such as isolating immune cells, stem cells, or tumor cells from biological samples.

In the field of medicine, "time factors" refer to the duration of symptoms or time elapsed since the onset of a medical condition, which can have significant implications for diagnosis and treatment. Understanding time factors is crucial in determining the progression of a disease, evaluating the effectiveness of treatments, and making critical decisions regarding patient care.

For example, in stroke management, "time is brain," meaning that rapid intervention within a specific time frame (usually within 4.5 hours) is essential to administering tissue plasminogen activator (tPA), a clot-busting drug that can minimize brain damage and improve patient outcomes. Similarly, in trauma care, the "golden hour" concept emphasizes the importance of providing definitive care within the first 60 minutes after injury to increase survival rates and reduce morbidity.

Time factors also play a role in monitoring the progression of chronic conditions like diabetes or heart disease, where regular follow-ups and assessments help determine appropriate treatment adjustments and prevent complications. In infectious diseases, time factors are crucial for initiating antibiotic therapy and identifying potential outbreaks to control their spread.

Overall, "time factors" encompass the significance of recognizing and acting promptly in various medical scenarios to optimize patient outcomes and provide effective care.

Bone substitutes are materials that are used to replace missing or damaged bone in the body. They can be made from a variety of materials, including natural bone from other parts of the body or from animals, synthetic materials, or a combination of both. The goal of using bone substitutes is to provide structural support and promote the growth of new bone tissue.

Bone substitutes are often used in dental, orthopedic, and craniofacial surgery to help repair defects caused by trauma, tumors, or congenital abnormalities. They can also be used to augment bone volume in procedures such as spinal fusion or joint replacement.

There are several types of bone substitutes available, including:

1. Autografts: Bone taken from another part of the patient's body, such as the hip or pelvis.
2. Allografts: Bone taken from a deceased donor and processed to remove any cells and infectious materials.
3. Xenografts: Bone from an animal source, typically bovine or porcine, that has been processed to remove any cells and infectious materials.
4. Synthetic bone substitutes: Materials such as calcium phosphate ceramics, bioactive glass, and polymer-based materials that are designed to mimic the properties of natural bone.

The choice of bone substitute material depends on several factors, including the size and location of the defect, the patient's medical history, and the surgeon's preference. It is important to note that while bone substitutes can provide structural support and promote new bone growth, they may not have the same strength or durability as natural bone. Therefore, they may not be suitable for all applications, particularly those that require high load-bearing capacity.

Metabolic bone diseases are a group of conditions that affect the bones and are caused by disorders in the body's metabolism. These disorders can result in changes to the bone structure, density, and strength, leading to an increased risk of fractures and other complications. Some common examples of metabolic bone diseases include:

1. Osteoporosis: a condition characterized by weak and brittle bones that are more likely to break, often as a result of age-related bone loss or hormonal changes.
2. Paget's disease of bone: a chronic disorder that causes abnormal bone growth and deformities, leading to fragile and enlarged bones.
3. Osteomalacia: a condition caused by a lack of vitamin D or problems with the body's ability to absorb it, resulting in weak and soft bones.
4. Hyperparathyroidism: a hormonal disorder that causes too much parathyroid hormone to be produced, leading to bone loss and other complications.
5. Hypoparathyroidism: a hormonal disorder that results in low levels of parathyroid hormone, causing weak and brittle bones.
6. Renal osteodystrophy: a group of bone disorders that occur as a result of chronic kidney disease, including osteomalacia, osteoporosis, and high turnover bone disease.

Treatment for metabolic bone diseases may include medications to improve bone density and strength, dietary changes, exercise, and lifestyle modifications. In some cases, surgery may be necessary to correct bone deformities or fractures.

Megakaryocytes are large, specialized bone marrow cells that are responsible for the production and release of platelets (also known as thrombocytes) into the bloodstream. Platelets play an essential role in blood clotting and hemostasis, helping to prevent excessive bleeding during injuries or trauma.

Megakaryocytes have a unique structure with multilobed nuclei and abundant cytoplasm rich in organelles called alpha-granules and dense granules, which store various proteins, growth factors, and enzymes necessary for platelet function. As megakaryocytes mature, they extend long cytoplasmic processes called proplatelets into the bone marrow sinuses, where these extensions fragment into individual platelets that are released into circulation.

Abnormalities in megakaryocyte number, size, or function can lead to various hematological disorders, such as thrombocytopenia (low platelet count), thrombocytosis (high platelet count), and certain types of leukemia.

Mucopolysaccharidosis I (MPS I) is a rare genetic disorder caused by the deficiency of an enzyme called alpha-L-iduronidase. This enzyme is responsible for breaking down complex sugars called glycosaminoglycans (GAGs), also known as mucopolysaccharides, in the body.

When the enzyme is deficient, GAGs accumulate in various tissues and organs, leading to a range of symptoms that can affect different parts of the body, including the skeletal system, heart, respiratory system, eyes, and central nervous system. There are three subtypes of MPS I: Hurler syndrome (the most severe form), Hurler-Scheie syndrome (an intermediate form), and Scheie syndrome (the least severe form).

The symptoms and severity of MPS I can vary widely depending on the specific subtype, with Hurler syndrome typically causing more significant health problems and a shorter life expectancy than the other two forms. Treatment options for MPS I include enzyme replacement therapy, bone marrow transplantation, and various supportive therapies to manage symptoms and improve quality of life.

Iduronidase is a type of enzyme that helps break down complex sugars called glycosaminoglycans (GAGs) in the body. Specifically, iduronidase is responsible for breaking down a type of GAG called dermatan sulfate and heparan sulfate.

Deficiency or absence of this enzyme can lead to a genetic disorder known as Mucopolysaccharidosis Type I (MPS I), which is characterized by the accumulation of GAGs in various tissues and organs, leading to progressive damage and impairment. There are two forms of MPS I: Hurler syndrome, which is the severe form, and Scheie syndrome, which is the milder form.

Iduronidase replacement therapy is available for the treatment of MPS I, in which the missing enzyme is delivered directly to the patient's body through intravenous infusion. This helps break down the accumulated GAGs and prevent further damage to the tissues and organs.

Mucopolysaccharidosis VI (MPS VI), also known as Maroteaux-Lamy syndrome, is a rare genetic disorder caused by the deficiency of an enzyme called N-acetylgalactosamine 4-sulfatase. This enzyme is responsible for breaking down complex sugars called glycosaminoglycans (GAGs) or mucopolysaccharides, which are found in various tissues and organs throughout the body.

When the enzyme is deficient, GAGs accumulate within the lysosomes of cells, leading to cellular dysfunction and tissue damage. This accumulation results in a range of symptoms that can affect multiple organ systems, including the skeletal system, cardiovascular system, respiratory system, and central nervous system.

The signs and symptoms of MPS VI can vary widely among affected individuals, but common features include: coarse facial features, short stature, stiff joints, restricted mobility, recurrent respiratory infections, hearing loss, heart valve abnormalities, and clouding of the cornea. The severity of the disease can range from mild to severe, and life expectancy is generally reduced in individuals with more severe forms of the disorder.

MPS VI is inherited as an autosomal recessive trait, which means that an individual must inherit two copies of the mutated gene (one from each parent) to develop the condition.

Mucopolysaccharidosis (MPS) VII, also known as Sly syndrome, is a rare genetic disorder caused by the deficiency of the enzyme beta-glucuronidase. This enzyme is responsible for breaking down complex sugars called glycosaminoglycans (GAGs), or mucopolysaccharides, in the body. When this enzyme is not present in sufficient amounts, GAGs accumulate in various tissues and organs, leading to progressive damage.

The symptoms of MPS VII can vary widely, but often include coarse facial features, short stature, skeletal abnormalities, hearing loss, heart problems, and intellectual disability. Some individuals with MPS VII may also have cloudy corneas, enlarged liver and spleen, and difficulty breathing due to airway obstruction. The severity of the condition can range from mild to severe, and life expectancy is often reduced in those with more severe symptoms.

MPS VII is inherited in an autosomal recessive manner, which means that an individual must inherit two copies of the mutated gene (one from each parent) in order to develop the condition. Treatment for MPS VII typically involves enzyme replacement therapy, which can help to slow down the progression of the disease and improve some symptoms. However, there is currently no cure for this condition.

Mucopolysaccharidosis III, also known as Sanfilippo syndrome, is a genetic disorder caused by the deficiency of specific enzymes needed to break down complex sugar molecules called glycosaminoglycans (GAGs) or mucopolysaccharides. This results in an accumulation of these substances in various tissues and organs, leading to progressive damage.

There are four main types of Mucopolysaccharidosis III (A, B, C, and D), each caused by a deficiency in one of the following enzymes: heparan N-sulfatase (type A), alpha-N-acetylglucosaminidase (type B), acetyl-CoAlpha-glucosaminide acetyltransferase (type C), or N-acetylglucosamine 6-sulfatase (type D).

The symptoms of Mucopolysaccharidosis III typically become apparent between the ages of 2 and 6, and may include developmental delays, hyperactivity, behavioral problems, sleep disturbances, coarse facial features, hirsutism, hepatosplenomegaly (enlarged liver and spleen), and joint stiffness. Over time, individuals with Mucopolysaccharidosis III may experience a decline in cognitive abilities, loss of previously acquired skills, and mobility issues.

Currently, there is no cure for Mucopolysaccharidosis III, and treatment is focused on managing the symptoms and improving quality of life. Enzyme replacement therapy, gene therapy, and stem cell transplantation are some of the experimental treatments being investigated for this condition.

Enzyme Replacement Therapy (ERT) is a medical treatment approach in which functional copies of a missing or deficient enzyme are introduced into the body to compensate for the lack of enzymatic activity caused by a genetic disorder. This therapy is primarily used to manage lysosomal storage diseases, such as Gaucher disease, Fabry disease, Pompe disease, and Mucopolysaccharidoses (MPS), among others.

In ERT, the required enzyme is produced recombinantly in a laboratory using biotechnological methods. The purified enzyme is then administered to the patient intravenously at regular intervals. Once inside the body, the exogenous enzyme is taken up by cells, particularly those affected by the disorder, and helps restore normal cellular functions by participating in essential metabolic pathways.

ERT aims to alleviate disease symptoms, slow down disease progression, improve quality of life, and increase survival rates for patients with lysosomal storage disorders. However, it does not cure the underlying genetic defect responsible for the enzyme deficiency.

Some malignancies that have spread to involve the bone marrow, such as leukemia or advanced Hodgkin's disease, also cause ... Weiss RB, Brunning RD, Kennedy BJ (December 1975). "Hodgkin's disease in the bone marrow". Cancer. 36 (6): 2077-83. doi:10.1002 ... The SARS disease caused lymphocytopenia. Among patients with laboratory-confirmed COVID-19 in Wuhan China through January 29th ... Guan, Wei-jie (February 28, 2020). "Clinical Characteristics of Coronavirus Disease 2019 in China". The New England Journal of ...
Bone marrow disease: like Paroxysmal Nocturnal Hemoglobinuria 4. Sickle Cell Anemia's, 5. Thalassemia 6.Immune Deficiencies ... The use of blood stem cells has emerged as a potentially curative option for the treatment of several diseases, including blood ... For example - Diabetes, Brain injuries etc disease. Officially opened on 28 September 2005 by Health Minister Mr. Khaw Boon Wan ... 1. Leukemia's: such as Acute Myelogenous Leukemia 2. Lymphomas: non hodking's Lymphoma, Lymph proliferative disease 3. ...
Picard, Andre (10 December 2012). "Bone-marrow transplant cures girl's inflammatory bowel disease". The Globe and Mail. Toronto ... personalized treatment to be given to children with genetic forms of intestinal disease, including bone marrow transplantation ... He has uncovered the underlying genetic and functional causes of a number of diseases, including PLVAP, ARPC1B and SYK. Muise's ... He was awarded a Tier 1 Canada Research Chair in pediatric inflammatory bowel disease in 2019. Muise's clinical and research ...
"Paediatric Immunology, Bone Marrow Transplantation and Infectious Diseases". Newcastle Hospitals. Retrieved 15 April 2018. "The ... The Great North Children's Hospital is one of two units in the UK which perform bone marrow transplants for children who were ...
"Bone Marrow Disease Claims Life of Ex-Parkettes' Coach". The Morning Call. Allentown, Pennsylvania. October 20, 1998. Retrieved ... On October 18, 1998, Babcock passed away due to complications from a bone marrow transplant to fight Myelodysplastic syndrome ... "Former National Champion Brian Babcock Loses Battle with Blood Disease". usagym.org. October 19, 1998. Retrieved September 5, ...
In September 2012 Pluristem reported saving the life of a third bone marrow disease patient using its PLacental eXpanded cell ... "Pluristem stem cells save 3rd bone marrow disease patient". Globes. 5 September 2012. Retrieved 10 September 2012. Press, Viva ... cells were injected into the muscles of a 7-year-old Romanian girl undergoing treatment for bone marrow aplasia disease at the ... Results from the study suggest that PLX-R18 is safe and may significantly improve outcomes after bone marrow failure or ...
... transplants can be conducted to treat severe diseases of the bone marrow, including certain forms of cancer such as ... Bone marrow examination is the pathologic analysis of samples of bone marrow obtained via biopsy and bone marrow aspiration. ... To diagnose diseases involving the bone marrow, a bone marrow aspiration is sometimes performed. This typically involves using ... In adult humans, bone marrow is primarily located in the ribs, vertebrae, sternum, and bones of the pelvis. Bone marrow ...
He had been suffering from bone marrow disease for two years. He was laid to rest at the Zincirlikuyu Cemetery following the ...
"Allogeneic bone marrow transplantation for Alexander's disease". Bone Marrow Transplant. 20 (3): 247-9. doi:10.1038/sj.bmt. ... alexander_disease at NINDS "Cause of brain disease found". January 2, 2001 - via news.bbc.co.uk. "Alexander Disease - United ... Wikimedia Commons has media related to Alexander disease. OMIM entries on Alexander disease Infantile-onset Alexander disease ... A bone marrow transplant has been attempted on a child, but it made no improvement. Hydrocephalus may be seen in younger ...
Nakao K, Fujioka S (1968). "Thymidine kinase activity in the human bone marrow from various blood diseases". Life Sciences. 7 ( ... Wickramasinghe SN, Olsen I, Saunders JE (1975). "Thymidine kinase activity in human bone marrow cells". Scandinavian Journal of ... This disease has a wide range of aggressivity, from slow-growing indolent disease that hardly requires treatment to highly ... in peripheral lymphocytes during monocytosis and in bone marrow during pernicious anemia. As TK1 is present in cells during ...
Nakao K, Fujioka S (April 1968). "Thymidine kinase activity in the human bone marrow from various blood diseases". Life ... Wickramasinghe SN, Olsen I, Saunders JE (September 1975). "Thymidine kinase activity in human bone marrow cells". Scandinavian ... in peripheric lymphocytes during monocytosis and in bone marrow during pernicious anemia. As TK1 is present in cells during ... Enzymes of thymidine and thymidylate metabolism in normal and pathological blood and bone marrow cells]". Blut (in German). 25 ...
... some people still develop diseases because of this interference with bone marrow. Although people receiving chemotherapy are ... ISBN 978-1-60795-014-1. Krumbhaar EB (1919). "Role of the blood and the bone marrow in certain forms of gas poisoning". JAMA. ... The drug is given before chemotherapy to protect bone marrow function. Due to immune system suppression, neutropenic ... Chemotherapeutic drugs may be used at high doses to permanently remove the recipient's bone marrow cells (myeloablative ...
749 Those who are at risk of developing graft-versus-host disease, such as bone marrow transplant recipients, receive blood ... "Abo Compatibility and Acute Graft-Versus-Host Disease Following Allogeneic Bone Marrow Transplantation". Transplantation. 45 (6 ... if they have received a bone marrow or stem cell transplant from someone with a different blood type, or in patients with ... If IgG is bound to red blood cells in vivo, as may occur in autoimmune hemolytic anemia, hemolytic disease of the newborn and ...
Bob DeWeese died on November 20, 1990 from bone marrow disease in Billings. He continued making art up until his death. Montana ...
These landmark publications have been the basis for the new field of autologous bone marrow stem cell therapy for heart disease ... Int J Cardiol (2013). "Autologous bone marrow-derived stem cell therapy in heart disease: Discrepancies and contradictions". ... Strauer, Bodo; Brehm M.; Zeus T (2002). "Repair of infarcted myocardium by autologous intracoronary mononuclear bone marrow ... own bone marrow cells into the coronary arteries can increase the pumping efficacy of a weak heart. ...
"Autologous bone marrow-derived stem cell therapy in heart disease: Discrepancies and contradictions". International Journal of ... Bone marrow cells regenerate infarcted myocardium. Nature, 2001; 5:410 (6829):701-5. Strauer BE, Brehm M, Zeus T, Kostering M, ... Hernandez A, Sorg RV, Kogler G and Wernet P. Repair of infarcted myocardium by autologous intracoronary mononuclear bone marrow ... have started to appear in peer reviewed journals and a paper has been published that has shown the potential of bone marrow ...
Dogs taking colchicine must be monitored closely for signs of bone marrow disease. The prognosis is guarded for Shar Pei that ... Kidney and liver failure cannot be treated except by the conventional manner usually used for those diseases. Prevention of ... Articles needing additional references from January 2012, All articles needing additional references, Dog diseases). ...
"Abo Compatibility and Acute Graft-Versus-Host Disease Following Allogeneic Bone Marrow Transplantation". Transplantation. 45 (6 ... as it may be responsible for some cases of acute graft-versus-host disease. Other human blood group systems than ABO and Rh ... which could put them at risk for developing hemolytic disease of the newborn during pregnancy. When needing to give red blood ... because incompatibility puts the baby at risk for developing hemolytic disease of the newborn. It is also used before ...
He died in November 2006 from primary systemic amyloidosis, an incurable bone marrow disease. Raw Meet (Intakt, 2004) with ... Deaths from skeletal disease, 20th-century American male musicians, American male jazz musicians, Machine Gun (band) members, ...
"The Use of Nitrogen Mustard in Neoplastic Diseases of the Bone Marrow," Rev. Med. Liban. I (1961?): 45-51. (with E. Stephan) " ... with G.I. Abu-Haydar and N.A. Abu-Haydar) "Thalassemia Hemoglobin E. Disease. A Case Report from Quatar." Persian Gulf, Man., ... with N.A. Abu-Haydar) "Sickle Cell Disease in Lebanon and Syria." Acta Haemat., Basel XXVII (1962):268-273. ( ...
At age nine, he was diagnosed with aplastic anemia, a rare bone marrow disease. This became the subject of his one-man stand-up ...
Some ultimate effects of this disease include bone-marrow failure as well as cancer. This syndrome has been shown to arise from ... Spinal muscular atrophy affects up to 1 in 6,000 people and is the second leading cause of neuromuscular disease, after ... RNPs we have been able to better understand many important diseases. Spinal muscular atrophy - Mutations in the survival motor ...
... inflammatory responses within the bone marrow are believed to foster many hematological diseases. The secretion of IL-6 by bone ... July 1994). "Bone marrow angiogenesis and progression in multiple myeloma". Br. J. Haematol. 87 (3): 503-8. doi:10.1111/j.1365- ... They discovered increased bone marrow angiogenesis correlates with myeloma growth and supporting stromal cells are a ... The primary use of IMiDs in medicine is in the treatment of cancers and autoimmune diseases (including one that is a response ...
Albert Niemann (1880-1921). Pick's cell: Histiocyte found in the spleen and bone marrow in Niemann-Pick disease. It is similar ... ISBN 0-930405-26-9. Medicine.net Definition of Niemann-Pick disease Ludwig Pick at Who Named It Media related to Ludwig Pick ( ... Ludwig Pick made several contributions to academic pathology, particularly in the field of genitourinary diseases and the study ... Die Skelettform-ossuäre Form des Morbus Gaucher, 1927 - The skeletal form in regards to Gaucher's disease. Der Paratyphus, 1928 ...
... chronic granulomatous disease). In addition, diseases affecting the bone marrow may result in abnormal or few leukocytes. ... Diabetes, cardiovascular disease, allergies, and chronic obstructive pulmonary disease (COPD) are examples of diseases mediated ... Acne vulgaris Asthma Autoimmune diseases Autoinflammatory diseases Celiac disease Chronic prostatitis Colitis Diverticulitis ... Non-immune diseases with causal origins in inflammatory processes include cancer, atherosclerosis, and ischemic heart disease. ...
... is a fatal disease that causes exudative retinopathy and bone marrow failure. Other symptoms include severe ... There is no treatment for this disease yet.[citation needed] Revesz syndrome has so far been observed only in children. There ... Revesz syndrome is a genetic disease thought to be caused by short telomeres. Patients with Revesz syndrome have presented with ... is not much information about the disease because of its low frequency in general population and under reporting of cases.[ ...
"Robert Power's career with Orica-GreenEdge on hold due to rare bone marrow disease". 25 November 2015. http://www.cyclingquotes ... In November 2015 he was diagnosed with a rare form of bone marrow edema that meant he only made his Orica-BikeExchange debut in ...
Inherited bone marrow failure is often the cause in young children, while older children and adults may acquire the disease ... A maturation defect in genes is a common cause of inherited bone marrow failure. The most common cause of acquired bone marrow ... Chronic fatigue, shortness of breath, and recurrent colds can also be symptoms of bone marrow failure. Bone marrow failure in ... "Bone Marrow Failure In Children". Thomson Reuters (2011): 1-5. Retrieved 7 Nov 2011. Besa, Emmanuel C. "Bone Marrow Failure". ...
CXCR4 mutations cause symptomatic hyperviscosity syndrome and high bone marrow activity characteristic of the disease. However ... A bone marrow biopsy provides a sample of bone marrow, usually from the lower back of the pelvis bone. The sample is extracted ... "Allogeneic bone marrow transplantation for advanced Waldenstrom's macroglobulinemia". Bone Marrow Transplant. 23 (7): 747-9. ... Bone marrow tumour cells express the following antigen targets CD20 (98.3%), CD22 (88.3%), CD40 (83.3%), CD52 (77.4%), IgM ( ...
Richard Kiley, 76, American Emmy Award-winning film, television and stage actor, bone marrow disease. Graham Armitage, 62, ... Eddie Dean, 91, American country singer-songwriter and actor, heart and lung disease. Fritz Honegger, 81, Swiss politician. ... Kirk Alyn, 88, American actor (Superman, Blackhawk, Call of the Rockies), Alzheimer's disease. Tex Blaisdell, 78, American ... Bob Cato, 76, American photographer and graphic designer, Alzheimer's disease. Joseph DePietro, 84, American weightlifter, 1948 ...
Health Information on Bone Marrow Diseases: MedlinePlus Multiple Languages Collection ... Bone Marrow Diseases: MedlinePlus Health Topic - English Enfermedades de la médula ósea: Tema de salud de MedlinePlus - español ... Bone Marrow Biopsy - 简体中文 (Chinese, Simplified (Mandarin dialect)) Bilingual PDF ... Bone Marrow Biopsy - 繁體中文 (Chinese, Traditional (Cantonese dialect)) Bilingual PDF ...
Create healthcare diagrams like this example called Diseases Treatable with a Bone Marrow or Cord Blood Transplant in minutes ... Diseases Treatable with a Bone Marrow or Cord Blood Transplant. Create healthcare diagrams like this example called Diseases ... Bone Marrow/ Cord Blood Transplant. Sickle cell disease and thalassemia Severe aplastic anemia and other marrow failure states ... Diseases Treatable with a Bone Marrow or Cord Blood Transplant. Familial erythrophagocytic lymphohistiocytosis and other ...
General availability of bone marrow transplantation in the public health system (Noncommunicable diseases). This indicator is ... General availability of bone marrow transplantation in the public health system (Noncommunicable diseases) ...
Leptotrichia trevisanii Sepsis after Bone Marrow Transplantation. Emerging Infectious Diseases. 2013;19(10):1690-1691. doi: ... Risk factors for anaerobic bloodstream infections in bone marrow transplant recipients. Clin Infect Dis. 2001;33:338-43. DOI ... We have seen an increase in the number of bone marrow transplants performed, but there has been no major change in ... Our findings suggest routine use of 16S rRNA gene sequencing and increased numbers of bone marrow transplants as the major ...
Cancer after bone marrow transplantation. IBMTR and EBMT/EULEP Study Group on Late Effects. Bone Marrow Transplant. 1992. 10 ... Physical Complications After Bone Marrow Transplantation. *Psychosocial and Cognitive Complications of Bone Marrow ... In a study from the United States, long-term survivors of pediatric bone marrow transplantation followed in the Bone Marrow ... Long-term outcome of patients given transplants of mobilized blood or bone marrow: A report from the International Bone Marrow ...
Re-establishing the regenerative potential is the key to cure these diseases by regenerative medicine. Multipotent stem cells ... Their intrinsic regenerative capacities are disturbed in many hematological and musculoskeletal diseases. ... Blood and bone - conjoined twins in health and disease: bone marrow analogs for hematological and musculoskeletal diseases. ... This versatility will enable us to create biomimetic human in vitro models of the human bone marrow in health and disease, ...
Many of these cells originate from the bone marrow, and the beta-amyloid-40 and -42 i … ... of highly ramified and elongated microglia within the core of amyloid plaques in transgenic mouse models of Alzheimers disease ... Bone marrow-derived microglia play a critical role in restricting senile plaque formation in Alzheimers disease Neuron. 2006 ... These bone marrow-derived microglia are thus very efficient in restricting amyloid deposits. Therapeutic strategies aiming to ...
A Histochemical Study of Alkaline Phosphatase in the Leukocytes of Blood and Bone Marrow in Various Diseases Subject Area: ... A Histochemical Study of Alkaline Phosphatase in the Leukocytes of Blood and Bone Marrow in Various Diseases. Acta Haematol 1 ...
Comparison of the diagnostic value of bone marrow biopsy and bone marrow aspiration in neoplastic disease ... Comparison of the diagnostic value of bone marrow biopsy and bone marrow aspiration in neoplastic disease ... We have examined the bone marrow biopsies and aspirates with respect to the adequacy of the bone marrow biopsy specimen, the ... The Jamshidi-Swaim biopsy needle was utilized to perform 205 bone marrow biopsies, accompanied by simultaneous bone marrow ...
Filed Under: Research & Development, Stem Cells Tagged With: bone marrow, bone marrow disease, medtech, University of ... Engineered tissue could eliminate radiation for bone marrow transplants. May 15, 2017. By Danielle Kirsh ... San Diego engineers have created artificial bone tissue that could eliminate the need for radiation before bone marrow ... led a team to develop a bone-like implant to eliminate the pre-treatment radiation that kills stem cells in a patients bone ...
Bone marrow is the spongy tissue inside some of your bones, such as your hip and thigh bones. It contains stem cells. The stem ... Occurrence of Bone Marrow Diseases. Number of Cases. The following are the number of Bone Marrow Diseases cases seen each year ... Common Causes of Bone Marrow Diseases. The following are the most common causes of Bone Marrow Diseases: *genetic mutation in ... Symptoms of Bone Marrow Diseases. The following features are indicative of Bone Marrow Diseases: *pain or fullness below the ...
However, how the fibrotic bone marrow niche itself impacts the function of monocytes and inflammation in the bone marrow was ... Deconstructing the Mechanics of Bone Marrow Disease. by Katherine Unger Baillie. Acollaborative team developed an alginate- ... a mechanical checkpoint that could be disrupted in myelofibrotic bone marrow and also may be at play in other fibrotic diseases ... myelofibrosis is a mechano-related disease that could be treated by interfering with the mechanical signaling in bone marrow ...
Psychosocial outcomes of bone marrow transplant for individuals affected by Mucopolysaccharidosis I Hurler Disease: Patient ... Psychosocial outcomes of bone marrow transplant for individuals affected by Mucopolysaccharidosis I Hurler Disease : Patient ... title = "Psychosocial outcomes of bone marrow transplant for individuals affected by Mucopolysaccharidosis I Hurler Disease: ... T1 - Psychosocial outcomes of bone marrow transplant for individuals affected by Mucopolysaccharidosis I Hurler Disease ...
More bone marrow transplants can be performed with 50% gene tissue matches. ... Sickle Cell Disease Investigating bone marrow transplants: A cure for some sickle cell disease patients. ... Sickle cell disease (SCD) specialists have performed bone marrow transplants in children since the 1990s. This procedure is the ... Its an exciting time for sickle cell disease research. Not everyone can get a bone marrow transplant. However, there are a lot ...
New bone marrow model could help people with sickle cell disease A new model showing how sickle cell blood cells operate within ... bone marrow is presented at a conference in Austria today. Researchers at Imperial College London, who developed the model, ... In the second year of a Northern California-based survey conducted by Sutter Health on women and heart disease, results show ... Tissue engineers can choose from a wide range of living cells, biomaterials and proteins to repair a bone defect. But finding ...
Bone Marrow Transplantation vs. Standard of Care in Patients With Severe Sickle Cell Disease ... This study is comparing long-term outcomes for patients who receive blood and bone marrow transplants and those who receive ... To participate in this study, you or your child must have congenital heart disease. This study is located in Los Angeles, Palo ... Find studies for diseases and conditions and see if you or a loved one is eligible. ...
Fulminant hepatic failure caused by adenovirus infection following bone marrow transplantation for Hodgkins disease. In: Bone ... Fulminant hepatic failure caused by adenovirus infection following bone marrow transplantation for Hodgkins disease. Bone ... Fulminant hepatic failure caused by adenovirus infection following bone marrow transplantation for Hodgkins disease, Bone ... Fulminant hepatic failure caused by adenovirus infection following bone marrow transplantation for Hodgkins disease. Bone ...
Half-matched bone marrow alleviates sickle cell disease. By Mali Wiederkehr , October 4, 2012 A recent study conducted at the ... demonstrated that bone marrow transplants only partially matched to a patients tissues can successfully eradicate the disease ... Hopkins School of Medicine provides renewed optimism for the treatment of sickle cell disease. Researchers have ...
Fibrotic lung diseases increase with age. bone tissue marrow lungs and. Fibrotic lung diseases increase with age. bone tissue ... 2.4 Analysis of Fibrocytes in Peripheral Blood Bone Marrow and Lung Cells from blood bone marrow and lung were surface stained ... Bone tissue marrow-derived fibrocytes had been cultured on matrices produced from Thy-1(+) or Thy-1(?) fibroblasts ± the pro- ... Mice were euthanized at 7 and 14 days following injury; lungs blood and bone marrow were harvested for analyses. 2.3 Lung ...
Some malignancies that have spread to involve the bone marrow, such as leukemia or advanced Hodgkins disease, also cause ... Weiss RB, Brunning RD, Kennedy BJ (December 1975). "Hodgkins disease in the bone marrow". Cancer. 36 (6): 2077-83. doi:10.1002 ... The SARS disease caused lymphocytopenia. Among patients with laboratory-confirmed COVID-19 in Wuhan China through January 29th ... Guan, Wei-jie (February 28, 2020). "Clinical Characteristics of Coronavirus Disease 2019 in China". The New England Journal of ...
Bone Marrow. If enough stem cells cant be retrieved from apheresis, they can be removed directly from the bone marrow. This ... Graft Versus Host Disease (GVHD): What You Need To Know About Having A Bone Marrow Transplant ... Graft Versus Host Disease (GVHD): What You Need To Know About Having A Bone Marrow Transplant. January 5, 2017 ... The marrow thats removed (harvested) passes through a series of filters to remove bone or tissue fragments and is then placed ...
Bone Marrow Derived CD34 + cells and Leukocytes in 729 Children and Adults with Non-malignant Diseases quantity. ... Bone Marrow Derived CD34 + cells and Leukocytes in 729 Children and Adults with Non-malignant Diseases. ... Home / Videos / Bone Marrow Derived CD34 + cells and Leukocytes in 729 Children and Adults with Non-malignant Diseases. ...
His bone marrow was characterized by marked vacuolization of myeloid precursor … ... Bone Marrow Diseases / complications * Bone Marrow Diseases / genetics* * Bone Marrow Diseases / pathology* ... His bone marrow was characterized by marked vacuolization of myeloid precursors and ringed sideroblasts. Autopsy examination ... Pearsons marrow/pancreas syndrome: a histological and genetic study Virchows Arch A Pathol Anat Histopathol. 1993;423(3):227- ...
CDC reports on a program to characterize epidemiology of sickle cell disease in two states ... CDC reports on a program to characterize epidemiology of sickle cell disease in two states ... Ashorobi D, Bhatt R. Bone marrow transplantation in sickle cell disease. In: StatPearls. Treasure Island, FL: StatPearls ... Sickle cell disease, unspecified. D57.1X. Sickle cell anemia without crisis. 282.61. Sickle cell/hemoglobin-SS disease without ...
Bone marrow (n=38) or peripheral blood mobilized donor mononuclear cells (n=12) were exposed ex vivo to CAMPATH-1 (IgM and ... Thirty-three patients (66%) remained disease free at a mean of 1,118 (median 1439; range 159-4,177) days. For all patients, the ... who had received marrow grafts from HLA-identical siblings. The conditioning regimen included six 2-Gy fractions of total body ... mainly because of the perceived increase in disease recurrence. METHODS: We retrospectively analyzed the outcome of 50 ...
Protein/Autologous Condition Serum, and Bone Marrow Aspirate. *Sundman, E. (Speaker). *Texas Tech University ... Biologic Therapy for Joint Disease Platelet-Rich Plasma, Interleukin-1 Receptor Antagonist. ...
Background: Hematopoietic cell transplantation (HCT) for sickle cell disease (SCD) is potentially curative, yet applied ... Results of a Multicenter Pilot Investigation of Bone Marrow Transplantation in Adults with Sickle Cell Disease (STRIDE). Blood ...
We were founded in memory of my sister Amber who passed away from Aplastic Anemia (rare bone marrow disease) after a 3.5 year ... Raising funds and awareness for children and families impacted by rare bone marrow diseases and blood disorders. ... charitable organization focused on raising funds and awareness for children and families impacted by rare bone marrow diseases ...
Molecular imaging, biodistribution and efficacy of mesenchymal bone marrow cell therapy in a mouse model of Chagas disease. ... biodistribution and efficacy of mesenchymal bone marrow cell therapy in a mouse model of Chagas disease. Together they form a ...
In this study, mesenchymal stem cells isolated from the bone-marrow of mice (BM MSCs), were labeled with Hoechst after low (3 ... Although an effective treatment for HD has remained elusive, current studies using transplants of bone-marrow-derived ... and rapidly develops symptoms analogous to the human form of the disease. It was observed that the transplanted cells survived ... Huntingtons disease (HD) is an autosomal dominant disorder caused by an expanded CAG repeat (greater than 38) on the short arm ...
  • In addition to complications seen from exposure to chemotherapy and radiation, patients undergoing allogeneic transplantation can experience unique late effects secondary to graft versus host disease (GVHD) and autoimmunity. (medscape.com)
  • In a study from the United States, long-term survivors of pediatric bone marrow transplantation followed in the Bone Marrow Transplant Survivors Study were compared with survivors of childhood cancer treated without bone marrow transplant from the Childhood Cancer Survivor Study. (medscape.com)
  • [ 2 , 8 ] Survivors of bone marrow transplantation were more likely to have a severe or life threatening condition (relative risk [RR] = 3.9), more than one chronic condition (RR = 2.6), functional impairment (RR=3.5), and activity limitations (RR = 5.8) than conventionally treated patients. (medscape.com)
  • These data reinforce the need for marked vigilance in ensuring proper screening and management of long-term survivors of bone marrow transplantation. (medscape.com)
  • Adenoviruses are increasingly realised to be responsible for serious morbidity and mortality following allogeneic bone marrow transplantation. (elsevierpure.com)
  • Bone Marrow Transplantation , 24 (1), 99-101. (elsevierpure.com)
  • BACKGROUND: Preventing graft-versus-host disease (GVHD) by depletion of T lymphocytes from the stem cell graft for transplantation remains controversial, mainly because of the perceived increase in disease recurrence. (ox.ac.uk)
  • Learn more at Be the Match , a leading bone marrow transplantation program that helps patients find life-saving donors and provides support throughout the transplant process. (cdc.gov)
  • Prochymal is a treatment for children suffering from graft-versus-host disease following bone marrow transplantation. (michaeljfox.org)
  • Create healthcare diagrams like this example called Diseases Treatable with a Bone Marrow or Cord Blood Transplant in minutes with SmartDraw. (smartdraw.com)
  • They might involve medicines, blood transfusions or a bone marrow transplant . (tabletwise.net)
  • Aim: To explore the frequency with which children and young people participate in social activities with peers, when they are affected by Mucopolysaccharidosis I Hurler Disease (MPS IH) post bone marrow transplant (BMT). (hud.ac.uk)
  • Since the 1990s, to do a bone marrow transplant, a patient had to have a sibling with a complete tissue match and take a medication that suppresses their immune system (Cyclophosphamide), as well as high dose chemotherapy. (medlineplus.gov)
  • Not everyone can get a bone marrow transplant. (medlineplus.gov)
  • We describe a case of fulminant hepatic failure due to adenovirus serotype 2 in a 39-year-old woman who received a matched sibling allogeneic bone marrow transplant for multiply relapsed Hodgkin's disease. (elsevierpure.com)
  • Allogeneic bone marrow transplant (BMT) is an essential treatment to cure patients with blood cancers such as leukemia. (medicalxpress.com)
  • Aaron is also a motivational speaker who inspires audiences with her story of being the first successful nonmatched bone marrow transplant for sickle cell disease. (cdc.gov)
  • The best bone marrow transplant outcomes happen when a patient's human leukocyte antigen (HLA) and that of a donor closely match. (cdc.gov)
  • The only known cure for sickle cell disease is a bone marrow transplant, and the procedure was still considered experimental. (cdc.gov)
  • Aaron successfully underwent the experimental nonmatch bone marrow transplant at age 12! (cdc.gov)
  • Our blood and bone marrow disease experts provide advanced transplant procedures for children and teenagers. (childrensnational.org)
  • Due to the limitations of such studies in human beings and animals, I propose to develop human in vitro models of healthy bone marrow, which can be induced to develop hematological and musculoskeletal diseases with high incidence, namely leukemia, multiple myeloma and bone metastasis. (europa.eu)
  • and multiple myeloma , disease of the bone marrow. (cancercare.org)
  • Now, a collaborative team from Penn , Harvard , the Dana-Farber Cancer Institute (DFCI), and Brigham and Women's Hospital has created a programmable hydrogel-based in vitro model mimicking healthy and fibrotic human bone marrow. (upenn.edu)
  • Centers for Disease Control and Prevention. (cdc.gov)
  • The Centers for Disease Control and Prevention (CDC) cannot attest to the accuracy of a non-federal website. (cdc.gov)
  • I'm Commander Ibad Khan and I'm representing the Clinician Outreach and Communication Activity, COCA, with the Emergency Risk Communication Branch at the Centers for Disease Control and Prevention. (cdc.gov)
  • Bone marrow is the spongy tissue inside some of your bones, such as your hip and thigh bones. (tabletwise.net)
  • Fibrosis can also occur in the bone marrow, the spongy tissue inside some bones that houses blood-producing hematopoietic stem cells (HSCs) and can lead to scarring and the disruption of normal functions. (upenn.edu)
  • Our findings suggest routine use of 16S rRNA gene sequencing and increased numbers of bone marrow transplants as the major reasons. (cdc.gov)
  • We have seen an increase in the number of bone marrow transplants performed, but there has been no major change in myeloablative regimens. (cdc.gov)
  • University of California San Diego engineers have created artificial bone tissue that could eliminate the need for radiation before bone marrow transplants. (drugdeliverybusiness.com)
  • Sickle cell disease (SCD) specialists have performed bone marrow transplants in children since the 1990s. (medlineplus.gov)
  • Bone marrow transplants are not working in 100% of patients. (medlineplus.gov)
  • Although an effective treatment for HD has remained elusive, current studies using transplants of bone-marrow-derived mesenchymal stem cells provides considerable promise. (biomedcentral.com)
  • Minimal residual disease negativity rates were 64.3% in peripheral blood and 35.7% in bone marrow (intent-to-treat analysis). (nature.com)
  • We were founded in memory of my sister Amber who passed away from Aplastic Anemia (rare bone marrow disease) after a 3.5 year battle. (lovingamber.com)
  • and immune-mediated diseases such as aplastic anemia, in which the bone marrow loses its ability to produce red-blood cells. (petmd.com)
  • There are thousands of people with sickle cell disease in the world, and a couple out there just like me," she said. (cdc.gov)
  • For the bone marrow aspiration, an EZIO-10 drill with 28mm bone marrow needle was used. (caringmedical.com)
  • In this setting, immediately perform a bone marrow aspiration and obtain a biopsy from the posterior iliac crest. (medscape.com)
  • Yes, Bone Marrow Diseases causes complications if it is not treated. (tabletwise.net)
  • To avoid some of these complications, use of adult, bone marrow (BM)-derived stem cells have gained considerable interest. (biomedcentral.com)
  • Sickle cell disease (SCD), an inherited blood disorder affecting an estimated 100,000 persons in the United States, is associated with multiple complications and reduced life expectancy. (cdc.gov)
  • Chronic blood cancers known as " myeloproliferative neoplasms " (MPNs) are one example, in which patients can develop fibrotic bone marrow, or myelofibrosis, that disrupts the normal production of blood cells. (upenn.edu)
  • The Jamshidi-Swaim biopsy needle was utilized to perform 205 bone marrow biopsies, accompanied by simultaneous bone marrow aspirates, on patients with lymphoma, leukaemia, and a variety of solid tumours. (bmj.com)
  • Then when we developed a half-matched protocol, half of the patients eventually rejected the cells, and the disease came back. (medlineplus.gov)
  • The NHLBI leads or sponsors studies for patients who have heart, lung, blood, or sleep related diseases or disorders. (nih.gov)
  • Every year, September 21 is observed as World Alzheimer's Day to raise awareness about the disease globally and challenge the stigma around it while empowering both caregivers and patients. (wn.com)
  • 2005). In addition, about half of patients with the closely related blood diseases, essential thrombocythemia (ET) and primary myelofibrosis (PMF), also carry the JAK21 m utation (Baxter et al. (cdc.gov)
  • Anemia is common in patients with chronic kidney disease. (medscape.com)
  • Multipotent stem cells of both tissues - hematopoietic stem cells (HSCs) for blood and mesenchymal stem/stromal (MSCs) for bone - are the basis for their regenerative capacity. (europa.eu)
  • This bone marrow film at 400X magnification demonstrates a complete absence of hemopoietic cells. (medscape.com)
  • Many of these cells originate from the bone marrow, and the beta-amyloid-40 and -42 isoforms are able to trigger this chemoattraction. (nih.gov)
  • Monocytes, a type of white blood cell belonging to the group of myeloid cells, are overproduced from HSCs in neoplasms and contribute to the inflammation in the bone marrow environment, or niche. (upenn.edu)
  • Combining this system with mouse in vivo models of myelofibrosis, the researchers demonstrated that monocytes decide whether to enter a pro-inflammatory state and go on to differentiate into inflammatory dendritic cells based on specific mechanical properties of the bone marrow niche with its densely packed ECM molecules. (upenn.edu)
  • It turns out that myelofibrosis is a mechano-related disease that could be treated by interfering with the mechanical signaling in bone marrow cells. (upenn.edu)
  • We are trying to create a state where instead of completely replacing bone marrow with that of their donors, we are creating a mixture of donor and patient cells because we now know that as low as 20% donor cells is enough to reverse sickle cell disease. (medlineplus.gov)
  • Tissue engineers can choose from a wide range of living cells, biomaterials and proteins to repair a bone defect. (news-medical.net)
  • A new model showing how sickle cell blood cells operate within bone marrow is presented at a conference in Austria today. (news-medical.net)
  • Ex vivo depletion of T cells from bone marrow grafts with CAMPATH-1 in acute leukemia: graft-versus-host disease and graft-versus-leukemia effect. (ox.ac.uk)
  • In this study, mesenchymal stem cells isolated from the bone-marrow of mice (BM MSCs), were labeled with Hoechst after low (3 to 8) or high (40 to 50) numbers of passages and then transplanted intrastriatally into 5-week-old R6/2 mice, which carries the N-terminal fragment of the human HD gene (145 to 155 repeats) and rapidly develops symptoms analogous to the human form of the disease. (biomedcentral.com)
  • most of the neutrophils in the body are contained in the bone marrow, either as mitotically active (one third) or postmitotic mature cells (two thirds). (medscape.com)
  • Medications for the underlying cause may be necessary, in addition to various medications to stimulate the production of neutrophils (a type of white blood-cell that fights infection), and another medication to stimulate the production of red-blood cells by bone marrow. (petmd.com)
  • Polycythemia vera (PV) is a rare blood disease in which the bone marrow makes too many red blood cells. (cdc.gov)
  • Non-Hodgkin Lymphomas Non-Hodgkin lymphomas are a heterogeneous group of disorders involving malignant monoclonal proliferation of lymphoid cells in lymphoreticular sites, including lymph nodes, bone marrow, the. (msdmanuals.com)
  • Whereas hypoxia in the individual with normal functioning kidneys leads to erythropoietin gene transcription, and hence increased RBC production, in those with anemia of chronic kidney disease, there is primary deficiency of erythropoietin production by the interstitial fibroblasts, also known as type I interstitial cells, thereby leading to anemia. (medscape.com)
  • This process causes many diseases in multiple organs, including lung fibrosis induced by smoking or asbestos, liver fibrosis induced by alcohol abuse, and heart fibrosis often following heart attacks. (upenn.edu)
  • However, how the fibrotic bone marrow niche itself impacts the function of monocytes and inflammation in the bone marrow was unknown. (upenn.edu)
  • Telomere shortening and chronic diseases could be caused by the same cell-damaging processes, such as oxidative stress and inflammation. (cdc.gov)
  • I wanted to develop a way to have half-match donors contribute bone marrow and for the outcomes to be successful. (medlineplus.gov)
  • Other possible tests include urine analysis, immunologic tests for infections diseases like feline leukemia virus (FeLV), and a bone marrow examination. (petmd.com)
  • The Loving Amber Foundation is a nonprofit 501(c)(3) charitable organization focused on raising funds and awareness for children and families impacted by rare bone marrow diseases and blood disorders. (lovingamber.com)
  • One way to fight diseases including HIV infection and autoimmune disorders could involve changing how a naturally occurring enzyme called SAMHD1 works to influence the immune system, new research suggests. (medicalxpress.com)
  • This battery of measurements are used in the diagnosis and treatment of certain liver, heart, and kidney diseases, acid-base imbalance in the respiratory and metabolic systems, other diseases involving lipid metabolism and various endocrine disorders as well as other metabolic or nutritional disorders. (cdc.gov)
  • Glycogen storage diseases (GSDs) are inherited disorders due to enzymatic defects that prevent breakdown of stored glycogen into glucose. (medscape.com)
  • Glycogen storage diseases ( GSD ) are a group of inherited autosomal recessive disorders caused by genetic mutations that lead to the inability to breakdown and metabolize glycogen into glucose. (medscape.com)
  • Exposure to benzene-contaminated toluene and bone marrow disorders - a retrospective exposure assessment. (cdc.gov)
  • Alanine aminotransferase measurements are used in the diagnosis and treatment of certain liver diseases (e.g., viral hepatitis and cirrhosis) and heart diseases. (cdc.gov)
  • Alkaline phosphatase measurements are used in the diagnosis and treatment of liver, bone, and parathyroid disease. (cdc.gov)
  • AST measurements are used in the diagnosis and treatment of certain types of liver and heart disease. (cdc.gov)
  • BUN measurements are used in the diagnosis of certain renal and metabolic diseases. (cdc.gov)
  • Calcium measurements are used in the diagnosis and treatment of parathyroid disease, bone diseases, chronic renal disease and tetany. (cdc.gov)
  • Creatinine measurements are useful in the diagnosis and treatment of renal diseases. (cdc.gov)
  • Iron (non-heme) measurements are used in the diagnosis and treatment of diseases such as iron deficiency anemia, chronic renal disease, and hemochromatosis (a disease associated with widespread deposit in the tissues of two iron-containing pigments, hemosiderin and hemofuscin, and characterized by pigmentation of the skin). (cdc.gov)
  • Some malignancies that have spread to involve the bone marrow, such as leukemia or advanced Hodgkin's disease, also cause lymphocytopenia. (wikipedia.org)
  • I'm hoping our research can go beyond sickle cell disease to have a bigger impact. (medlineplus.gov)
  • It's an exciting time for sickle cell disease research. (medlineplus.gov)
  • The number one disease among newborn babies is sickle cell disease. (medlineplus.gov)
  • Researchers at Imperial College London, who developed the model, hope that it will eventually be used by clinicians to advance more effective treatments for sickle cell disease. (news-medical.net)
  • A recent study conducted at the Hopkins School of Medicine provides renewed optimism for the treatment of sickle cell disease. (jhunewsletter.com)
  • Sickle Cell Disease is an awful genetic disease that disproportionally affects black people. (acsh.org)
  • It engaged a global discussion on sickle cell anemia, its perils and the advances in gene therapy that are showing great promise for this genetic disease. (acsh.org)
  • Did Gene Therapy Cure Sickle Cell Disease? (acsh.org)
  • A number of recent headlines imply that a new case study in the New England Journal of Medicine proves that gene therapy has cured sickle cell disease - a genetic disorder that causes tremendous pain, suffering and diminished life expectancy. (acsh.org)
  • Aaron was first diagnosed with sickle cell disease as an infant. (cdc.gov)
  • At the time, her parents were not aware of anyone else in her family who had sickle cell disease. (cdc.gov)
  • Her parents were frightened when Aaron was diagnosed because they didn't know much about sickle cell disease and were afraid they wouldn't know how to take care of her properly. (cdc.gov)
  • Having sickle cell disease nearly killed Aaron. (cdc.gov)
  • She was diagnosed with acute chest syndrome, a common complication of sickle cell disease that can result in lung injury, trouble breathing, low oxygen to the rest of the body and, possibly death. (cdc.gov)
  • Her sickle cell disease was cured. (cdc.gov)
  • Attending Camp New Hope, a summer camp for kids with sickle cell disease, helped Aaron make friends and realize that she was not alone. (cdc.gov)
  • Lymphocytes initially accumulate in the bone marrow and then spread to lymph nodes and other lymphoid tissues, eventually inducing splenomegaly, hepatomegaly, and systemic symptoms such as fatigue, fever, night sweats, early satiety, and unintentional weight loss. (msdmanuals.com)
  • Blood and bone are closely intertwined. (europa.eu)
  • The hypothesis of the current proposal is that only when taking both tissues and their mutual crosstalk into account, we will be able to understand how the regenerative potential of blood and bone is impaired in disease and how it can be re-established with novel treatment strategies. (europa.eu)
  • Tests for bone marrow diseases include blood and bone marrow tests. (tabletwise.net)
  • Pancytopenia does not actually refer to a disease, but rather to the simultaneous development of a number of blood-related deficiencies: non-regenerative anemia, leucopenia, and thrombocytopenia. (petmd.com)
  • The frequency of CBC checks depends on how severely low the cat's blood-cell and platelet counts were and are, as well as the underlying cause of the disease. (petmd.com)
  • Q: My friend says his uncle is being treated for a disease where he has too much blood and he gets "bloodletting'" every other day, like in the Middle Ages . (wn.com)
  • This is accomplished by removal of blood through periodic phlebotomy and drug treatment to suppress red blood cell production by the bone marrow. (cdc.gov)
  • In fact, in individuals with advanced stages of chronic kidney disease, the etiology of anemia tends to be multifactorial (eg, decreased RBC production due to lack of erythropoietin, increased RBC destruction due to hemolysis [intravascular or extravascular], as well as increased blood loss due to multiple venipunctures for an array of indications). (medscape.com)
  • Causes of bone marrow diseases include genetics and environmental factors. (tabletwise.net)
  • Elevated levels of the transaminases can indicate myocardial infarction, hepatic disease, muscular dystrophy, or organ damage. (cdc.gov)
  • It is currently the most sensitive enzymatic indicator of liver disease, with normal values rarely found in the presence of hepatic disease. (cdc.gov)
  • Von Gierke disease (GSD type Ia and Ib) was first reported in 1929 based on the autopsy findings in 2 children who had excessive hepatic and renal glycogen accumulation. (medscape.com)
  • 8. Chemotherapy is sometimes used to treat diseases other than cancers. (webmd.com)
  • Compiled for your convenience are articles on bone marrow failure research and treatment from the world's major journals on hematology / oncology. (aamds.org)
  • Biologic Therapy for Joint Disease Platelet-Rich Plasma, Interleukin-1 Receptor Antagonist. (ttu.edu)
  • Congenital Heart Disease GEnetic NEtwork Study (CHD GENES). (nih.gov)
  • This study will find both common genetic causes of congenital heart disease and ways that genes influence results of medical treatment. (nih.gov)
  • Epigenetic changes actually are more common than the genetic mutations long known to put people at risk for cancer and other diseases and they are probably inherited as well, Dr. Bhalla says. (sciencedaily.com)
  • While it is well established that HSCs are influenced by the bone marrow in their natural environment including MSCs and their progeny, surprisingly little attention has been paid to the reciprocal relationship. (europa.eu)
  • Serum elevations of ALT activity are rarely observed except in parenchymal liver disease, since ALT is a more liver-specific enzyme than aspartate aminotransferase (AST). (cdc.gov)
  • The morbidity and mortality depend greatly on the underlying etiology of the patient's anemia as well as the stage of the disease, whether early or advanced. (medscape.com)
  • It is possible that other mutations or predisposing factors are necessary for disease progression. (cdc.gov)
  • In some families, inheritance of exceptionally short telomeres is linked to specific diseases, such as pulmonary fibrosis or bone marrow failure. (cdc.gov)
  • The procedure is most often done on a pelvic or chest bone. (epnet.com)
  • Re-establishing the regenerative potential is the key to cure these diseases by regenerative medicine. (europa.eu)
  • The cutaneous porphyrias are dermatologic diseases that may or may not involve the liver and nervous system. (medscape.com)
  • Because it is a chronic disease people can go years without treatment, so it is important to monitor the disease for any changes. (cancercare.org)
  • GSD type I, also known as Von Gierke disease, is an autosomal recessive disorder, divided into two subtypes: type Ia and type Ib. (medscape.com)
  • Huntington's disease (HD) is an autosomal dominant disorder caused by an expanded CAG repeat (greater than 38) on the short arm of chromosome 4, resulting in loss and dysfunction of neurons in the neostriatum and cortex, leading to cognitive decline, motor dysfunction, and death, typically occurring 15 to 20 years after the onset of motor symptoms. (biomedcentral.com)
  • While arthroscopy with or without core decompression is the standard of care for unresolved cases of avascular necrosis of the talus, we present a case of avascular necrosis of the talar dome where symptoms resolved satisfactorily with direct bone marrow injections into structures into and around the ankle. (caringmedical.com)
  • Treatment depends on the primary condition that led to pancytopenia, as it is essential that the primary disease be diagnosed and treated first. (petmd.com)
  • A hollow biopsy needle will be inserted into the bone. (epnet.com)
  • This motion will allow a sample of bone marrow to enter the core of the needle. (epnet.com)
  • The bone marrow sample will be inside the needle. (epnet.com)
  • Here we show a massive infiltration of highly ramified and elongated microglia within the core of amyloid plaques in transgenic mouse models of Alzheimer's disease (AD). (nih.gov)
  • After 18 years of living with Alzheimer's disease , Marti Kaye spends most of her time in a wheelchair, expressionless. (wn.com)
  • Simple organic chemistry explains why this change profoundly affects those unfortunate enough to inherit the disease, which is characterized by abnormal hemoglobin. (acsh.org)
  • The focus of this report is the prevention of invasive pneumococcal disease (i.e., bacteremia, meningitis, or infection of other normally sterile sites) through the use of pneumococcal polysaccharide vaccine. (cdc.gov)
  • Researchers, journalists, and inquiring minds want to know more about telomeres, which seem to hold clues to human aging and age-related diseases. (cdc.gov)