• Histiocytic sarcoma complex (immunohistochemical features of dendritic cells, possibly interdigitating or perivascular DCs) 3.a. (wikipedia.org)
  • More recently, histiocytic diseases have been reclassified into five groups: (1) Langerhans-related, (2) cutaneous and mucocutaneous, (3) malignant histiocytosis, (4) Rosai-Dorfman disease, and (5) hemophagocytic lymphohistiocytosis and macrophage activation syndrome. (medscape.com)
  • Genotyping of 69 histiocytic lesions revealed that 23/48 Langerhans cell lesions were BRAF -V600E-mutant whereas all non-Langerhans cell lesions (including dendritic cell sarcoma, juvenile xanthogranuloma, Rosai-Dorfman disease, and granular cell tumor) were wild-type. (oncotarget.com)
  • In conclusion, BRAF mutations in histiocytic proliferations are restricted to lesions of the Langerhans-cell type. (oncotarget.com)
  • Langerhans cell histiocytosis (LCH) is a rare disease of unknown etiology, lacking an animal model, that cumulates symptoms that are found separately in various IL-17A-related diseases, such as aggressive chronic granuloma formation, bone resorption and soft tissue lesions with occasional neurodegeneration(11,12). (dtu.dk)
  • We examined IL-17A in the context of LCH and found that there were high serum levels of IL-17A during active LCH and unexpected IL-17A synthesis by dendritic cells (DCs), the major cell type in LCH lesions. (dtu.dk)
  • Arguments supporting the reactive nature of LCH include the occurrence of spontaneous remissions, the extensive elaboration of multiple cytokines by dendritic cells and T-cells (the so-called cytokine storm) in LCH lesions, and the good survival rate in patients without organ dysfunction. (medscape.com)
  • OBJECTIVES: To describe the morphologic characteristics of skin lesions, extent of extracutaneous disease, and outcomes in patients with neonatal presentation of Langerhans cell histiocytosis (LCH), and to examine clinical predictors of disease prognosis. (thedoctorsdoctor.com)
  • Langerhans cell histiocytosis (LCH) is a rare haematological neoplasm characterized by the accumulation of CD1a + , CD207/Langerin + histiocytes within inflammatory lesions. (biomedcentral.com)
  • Two decades later, with the advent of electron microscopy, Nezelof and colleagues identified a unique intracellular organelle, the Birbeck granule , in histiocytosis X lesions. (capsulehealth.one)
  • Hence cutaneous histiocytoma is a localized epidermal Langerhans cell tumor, and the rare examples of systemic spread of histiocytoma are best characterized as Langerhans cell histiocytosis (LCH) similar to that observed in humans. (wikipedia.org)
  • cell histiocytosis and giant cell tumor. (neobiotechnologies.com)
  • This classification attempts to classify lymphomas by cell type (i.e. the normal cell type that most closely resembles the tumor). (newworldencyclopedia.org)
  • A NET arises from cells that produce hormones, so the tumor can also produce hormones. (findzebra.com)
  • Epidermal invasion by cells of histiocytoma frequently occurs and intra-epidermal nests of histiocytes resemble Pautrier's aggregates, characteristically found in epidermotropic lymphoma (Mycosis Fungoides or MF). (wikipedia.org)
  • Histiocytomas lack expression of CD4 and Thy-1, which are consistently expressed by histiocytes in cutaneous and systemic histiocytosis. (wikipedia.org)
  • Class IIa dermal dendritic cell histiocytosis is a reactive increase in the number of non-Langerhans' cell histiocytes. (dermnetnz.org)
  • Histiocytosis is an umbrella term that refers to a relatively uncommon group of syndromes associated with the overproduction of certain infection-fighting white blood cells known as histiocytes. (tgh.org)
  • Langerhans' cell histiocytosis (LCH), previously known as histiocytosis X, describes a rare spectrum of conditions with abnormal proliferation of histiocytes, occuring in various locations throughout the body. (reviewofophthalmology.com)
  • Microscopic review of the tissue specimen revealed proliferation of histiocytes (left) with positive CD1a staining, a marker for Langerhans' cells (right). (reviewofophthalmology.com)
  • Idiopathic non-malignant disease characterized by idiopathic infiltration and accumulation of abnormal histiocytes (i.e. the Langerhans cells) within various tissues (bone marrow, skin, central nervous system, lung, liver, spleen, lymph nodes) causing focal or systemic effects. (capsulehealth.one)
  • I am an MRC Clinician Scientist and Honorary Consultant Paediatric Oncologist, providing a clinical service for young people with lymphoma or Langerhans cell histiocytosis at the Great North Children's Hospital, Newcastle. (ncl.ac.uk)
  • Our aim is to deeply characterise paediatric B-cell non-Hodgkin lymphoma to provide a comprehensive understanding of disease initiation and evolution in response to therapy. (ncl.ac.uk)
  • In lymphomas I have conducted studies in rare aggressive primary cutaneous tumors such as gamma/delta T cell lymphoma (expanding the description of the epidermotropic variant) as well as more indolent entities such as lymphomatoid papulosis (type D) and CD4-positive small to medium size T cell lymphoproliferative disorders. (mdanderson.org)
  • In lymphoma, the cells in the lymphatic system grow abnormally, dividing too rapidly and growing without any order or control (Longe 2005). (newworldencyclopedia.org)
  • DNA-microarray analysis of Burkitt's lymphoma and diffuse large B-cell lymphoma (DLBCL) showing differences in gene expression patterns. (newworldencyclopedia.org)
  • green indicates genes that are overexpressed in normal cells compared to lymphoma cells and red indicates genes that are overexpressed in lymphoma cells compared to normal cells. (newworldencyclopedia.org)
  • Acute lymphoblastic leukemia (ALL), Multiple myeloma (MM), and Chronic lymphocytic leukemia (CLL), and Diffuse Large B-Cell Lymphoma (DLBCL). (codemap.com)
  • Discordant lymphomas of classic Hodgkin lymphoma and peripheral T-cell lymphoma following dupilumab treatment for atopic dermatitis. (u-tokyo-hemat.com)
  • Notably, LCH cells have been found to express markers of both resting epidermal Langerhans cells (CD1a, intracellular major histocompatibility complex II [MHCII], Birbeck granules) and activated Langerhans cells (including CD54 and CD58). (medscape.com)
  • In immunohistochemical study, these eosinophilic cells expressed CD1a and S100 protein. (scirp.org)
  • LCH is characterized by the proliferation of CD1a-positive activated Langerhans (not atypical Langerhans cell, morphologically)-like cells (LCH cells) generating inflammatory granuloma. (mayabouchenaki.com)
  • Positive immunohistological staining with neuronal markers S100 and CD1a are specific for Langerhans' cells and help to confirm the diagnosis. (reviewofophthalmology.com)
  • CD1a is a protein of 43 to 49 kDa and is expressed on dendritic cells and cortical thymocytes. (micro-shop.pl)
  • CD1a [O10] staining has been shown to be useful in the differentiation of Langerhans cells from interdigitating cells. (micro-shop.pl)
  • Canine cutaneous histiocytoma (derived from specialised epidermic dendritic cells, the Langerhans cells) 2. (wikipedia.org)
  • Cutaneous histiocytosis (CH) 2.b. (wikipedia.org)
  • Multiple histiocytomas may look like cutaneous histiocytosis, although morphologically histiocytomas are consistently epidermotropic and commonly epidermally invasive, these are not features of cutaneous histiocytosis. (wikipedia.org)
  • Cutaneous histiocytoses are classified according to the type of histiocyte within the skin. (dermnetnz.org)
  • The photoconjugation of psoralens with DNA produces an antiproliferative reaction in the skin, generates programmed cell death (apoptosis), and induces down-regulation of the cutaneous immune system. (medscape.com)
  • The working group of the Histiocyte Society divided histocytic disorders into three groups: (1) dendritic cell histiocytosis, (2) macrophage-related disorders, and (3) malignant histiocytosis. (medscape.com)
  • Since patients with GPA have augmented neutrophil PR3 expression, and PR3-expressing apoptotic cells frustrate macrophage phagocytosis and cellular clearance, we investigated the role of PR3 in stimulating giant cell and granuloma formation. (bmj.com)
  • Frédéric Geissmann and his team have described the common precursor for macrophage, monocytes and dendritic cells (Fogg et al. (cerclefser.org)
  • These neoplastic cells are the result of sporadic activating mutations in genes of the mitogen-activated protein kinase (MAPK) signaling pathway expressed by multipotent hematopoietic stem/progenitor cells or committed myeloid precursors [ 1 ]. (biomedcentral.com)
  • Using a more sensitive technique for detection of mutant BRAF alleles (droplet digital PCR) [ 9 ], BRAF V600E was not detected in DNA extracted from myeloid or lymphoid cells isolated by flowcytometric sorting from a peripheral blood sample collected at the time of the fourth bone sample. (biomedcentral.com)
  • Notch signaling is a pathway that plays a role in the development and differentiation of many cell types, including myeloid cells. (upenn.edu)
  • Notch-dependent cooperativity between myeloid lineages refers to the process by which different myeloid cell types interact and cooperate with each other through the Notch signaling pathway. (upenn.edu)
  • Understanding how myeloid cells interact and cooperate through the Notch pathway can help researchers understand how the immune system functions and how to treat or prevent immune-related diseases. (upenn.edu)
  • The biopsy specimen from the right maxillary sinus showed soft tissue infiltration with myeloid leukemic cells, consistent with a chloroma. (contemporarypediatrics.com)
  • CCDC88C-FLT3 gene fusion in CD34-positive haematopoietic stem and multilineage cells in myeloid/lymphoid neoplasm with eosinophilia. (u-tokyo-hemat.com)
  • Epidermal Langerhans cells are unique among dendritic cells in that they arise not from myeloid progenitor cells in bone marrow but rather from yolk-sac progenitors and fetal liver-derived monocytes that populate the skin before birth and are maintained locally under steady-state conditions. (capsulehealth.one)
  • Other disorders similar to LCH include Histiocytoses Rosai-Dorfman, juvenile xanthogranuloma, and Erdheim-Chester disease but they do not share the exact phenotypic signature of LCH. (netlify.app)
  • We report a rare case of adult Langerhans cell histiocytosis in the dorsal spine causing a spinal cord compression associated with a pulmonary process treated by surgery, radiotherapy and systemic therapy with good evolution. (scirp.org)
  • Single-agent chemotherapy with cladribine (2-chlorodeoxyadenosine/2-CdA) may be a promising treatment for single-system pulmonary Langerhans cell histiocytosis. (medscape.com)
  • Гістіоцитоз легеневих клітин Лангерганса Pulmonary Langerhans cell histiocytosis (PLCH) is proliferation of monoclonal Langerhans cells in lung interstitium and airspaces. (msdmanuals.com)
  • On the other hand, the infiltration of organs by a monoclonal population of aberrant cells, the possibility of lethal evolution, and the cancer-based modalities of successful treatment are all consistent with a neoplastic process. (medscape.com)
  • Monoclonal antibodies, such as blinatumomab, may induce changes in the body's immune system and may interfere with the ability of cancer cells to grow and spread. (policylab.us)
  • Giving monoclonal antibody therapy with chemotherapy may kill more cancer cells. (policylab.us)
  • The antibody is a new monoclonal antibody against a C-type lectin found on the surface of Langerhans cells. (homeremedylifestyle.com)
  • In 1868, Paul Langerhans discovered the epidermal dendritic cells that now bear his name. (medscape.com)
  • Langerhans cells are named after Paul Langerhans , a bright, young medical student who worked with the new technique of gold colloid staining in the mid-19th century. (capsulehealth.one)
  • The marrow specimen showed extensive replacement of normal hematopoietic elements by immature monocytic cells. (contemporarypediatrics.com)
  • The ultrastructural hallmark of the Langerhans cell, the Birbeck granule, was described a century later. (medscape.com)
  • Specifically, a variety of other cellular populations have been identified that possess phenotypic characteristics similar to Langerhans cells, including expression of CD207 and Birbeck granules. (medscape.com)
  • Langerhans cell histiocytoses are all defined by the presence of a unique ultrastructural organelle, the Birbeck granule. (thedoctorsdoctor.com)
  • Dendritic cells in the skin are specifically called Langerhans cells, and these Langerhans cells have tennis-racquet shaped granules called Birbeck granules. (pixorize.com)
  • At this point, Birbeck granules were thought to be exclusive to epidermal Langerhans cells, skin-restricted cells of the mononuclear phagocyte system. (capsulehealth.one)
  • To better understand those processes, Frédéric Geissmann is investigating the differentiation and the function of those cells. (cerclefser.org)
  • This can involve the activation of Notch receptors on one cell type by ligands on another cell type, or the production of signaling molecules by one cell type that affect the differentiation or function of another cell type. (upenn.edu)
  • Although the structure of the granulomas and multinucleated giant cells in granulomatosis with polyangiitis (GPA) is known, little is established about what drove granuloma formation and why it is infrequently found in patients with myeloperoxidase-antineutrophil cytoplasm antibodies. (bmj.com)
  • Granulomas are exclusively found in GPA and form around multinucleated giant cells (MGCs), at sites of microabscesses, containing apoptotic and necrotic neutrophils. (bmj.com)
  • Systemic histiocytosis (SH) 3. (wikipedia.org)
  • Systemic Histiocytosis (SH) was originally recognized in closely related Bernese Mountain Dogs. (wikipedia.org)
  • We concluded that monocytes expressing the mutated form of Braf are the cell of origin of systemic LCH in mice. (histio.org)
  • Letterer-Siwe disease was described in infants with aggressive and generally fatal systemic disease, including skin, liver, spleen, and bone marrow infiltration by reticuloendothelial cells. (capsulehealth.one)
  • Normal histiocyte cells are originally formed within the bone marrow and then migrate throughout the body via the bloodstream. (dermnetnz.org)
  • Langerhans cells are dendritic cells that arise from bone marrow and migrate to skin. (osmosis.org)
  • If histiocytosis is suspected, a physician will usually order a tissue biopsy or a bone marrow aspiration to confirm the diagnosis. (tgh.org)
  • They are studying the cellular origin and the mechanisms that control the establishments and maintainance of these networks of 'resident' cell in skin and brain, i) whether they are renewed from bone marrow or 'niche' precursors, or if mature cells divide and self renew, ii) whether similar or separate mechanisms control their accumulation and activation during inflammation, and iii) their role in skin and brain inflammation. (cerclefser.org)
  • We found that transplant of bone marrow cells from a BRAF mouse into a normal recipient animal promoted development of a LCH-like disease in the liver, spleen and lung but not in the brain. (histio.org)
  • Sinus histiocytosis with massive lymphadenopathy (SHML), initially described in 1969 by Rosai and Dorfman,[ 22 ] is a rare, nonneoplastic lymphoproliferative disorder that is characterized by its histological features. (surgicalneurologyint.com)
  • HN - 2008 BX - Granulosa Cells, Cumulus MH - Coronary Sinus UI - D054326 MN - A07.231.908.194.500 MS - A short vein that collects about two thirds of the venous blood from the MYOCARDIUM and drains into the RIGHT ATRIUM. (bvsalud.org)
  • The term Langerhans cell histiocytosis is generally preferred to the older term, histiocytosis X. This newer name emphasizes the histogenesis of the condition by specifying the type of lesional cell and removes the connotation of the unknown ("X") because its cellular basis has now been clarified. (medscape.com)
  • Therefore, in addition to epidermal Langerhans cells, other potential cellular origins for LCH include dermal langerin + dendritic cells, lymphoid tissue-resident langerin + dendritic cells, and monocytes that can be induced by local environmental stimuli to acquire a Langerhans cell phenotype. (medscape.com)
  • This newer name emphasizes the histogenesis of the condition by specifying the type of lesional cell and removes the connotation of the unknown (\"X\") because its cellular basis has now been clarified. (mayabouchenaki.com)
  • One of the key cellular components with functional roles in both innate and adaptive arms of the immune response are Langerhans cells (LCs) (2). (mayabouchenaki.com)
  • The term "oncotarget" encompasses all molecules, pathways, cellular functions, cell types, and even tissues that can be viewed as targets relevant to cancer as well as other diseases. (oncotarget.com)
  • 2009), and investigated the pathophysiology of diseases of this cellular system, incliding Langerhans cell histiocytosis (Senechal et al. (cerclefser.org)
  • Methods We stimulated purified monocytes and whole peripheral blood mononuclear cells (PBMCs) from patients with GPA, patients with MPA or healthy controls with PR3 or MPO and visualised MGC and granuloma-like structure formation using light, confocal and electron microscopy, as well as measuring the cell cytokine production. (bmj.com)
  • Langerhans cell histiocytosis (LCH) and In LCH, overproduced, immature Langerhans cells cluster together and Cytokine pattern of Langerhans cells isolated from murine epidermal cell cultures. (netlify.app)
  • In 1868 , Langerhans described an epidermal cell population, accounting for approximately 1% of epidermal cells, with characteristic dendrites that he described as extracutaneous nerves. (capsulehealth.one)
  • I also work closely with UK and European colleagues (iBFM, EICNHL, ESID) to develop our understanding of development of lymphoid malignancy in children with inherited immune deficiency or DNA repair disorders and provide the paediatric clinical service for Langerhans cell histiocytosis and collaborate with Newcastle Human Dendritic Cell Laboratory ( http://www.hudendritic.org/ ) run by Professor Matt Collin. (ncl.ac.uk)
  • IL-17A is a T cell-specific cytokine(1) that is involved in chronic inflammations, such as Mycobacterium infection(2), Crohn's disease(3), rheumatoid arthritis(4) and multiple sclerosis(5). (dtu.dk)
  • A surgical biopsy showed an infiltrate of eosinophilic cells with oval, grooved and convoluted nucleus, associated with eosinophils, lymphocytes and plasma cells ( Figure 2 ). (scirp.org)
  • It consists of Langerhans cells with oval, grooved and convoluted nucleus and slightly eosinophilic cytoplasm, associated with eosinophils, lymphocytes and plasma cells (H & E × 400). (scirp.org)
  • This is because they have an abnormal increase in immune cells. (homeremedylifestyle.com)
  • This disorder is characterized by an abnormal increase in certain immune cells called histiocyte cells. (netlify.app)
  • Langerhans cell histiocytosis (LCH) is a disease characterized by the abnormal proliferation of leukocytes in the liver, lung and spleen. (histio.org)
  • In LCH, these cells become abnormal and form tumors in various parts of the body. (scitechdaily.com)
  • The abnormal cells in LCH have abnormal proliferation and lower antigen-presenting capability. (capsulehealth.one)
  • The study was funded by the Erik and Edith Fernström Foundation for Medical Research, the Swedish Childhood Cancer Foundation, the Histiocytosis Association, the VIVA Foundation for children with cancer, the Wellcome Trust, the CRUK Biomarker Project, Histio UK and Bright Red. (scitechdaily.com)
  • [ 3 ] Taken together, these findings have led some to speculate that LCH is not a specific disease of epidermal Langerhans cells, but rather one of mononuclear phagocyte dysregulation. (medscape.com)
  • Symptoms of Langerhans' Cell Histiocytosis vary depending on the part of the body affected and the severity of the disease. (homeremedylifestyle.com)
  • In the most severe form of the disease, the cancer cells can spread to other parts of the body. (homeremedylifestyle.com)
  • Langerhans' cell histiocytosis is a rare disease . (homeremedylifestyle.com)
  • The best way to treat Langerhans' cell histiocytosis is to have the disease treated by experts who understand the disease. (homeremedylifestyle.com)
  • Same as with "The Dungeon" and Single-celled organisms are organized using specialized cell organelles, while several cells may organize into larger structures, such as tissues and organ Single-celled organisms are organized using specialized cell organelles, while sever B cells are immune cells that provide protection against specific pathogens and disease through the production of antibodies. (netlify.app)
  • In this proposal we will define which cell is important for development of disease in the mouse model and how it causes disease. (histio.org)
  • Data from our lab showed that expression of the mutated form of Braf in cells of the monocytes induced LCH-like disease in mice (these animals are called FRBRAF mice). (histio.org)
  • Ironically, the lymphatic system is fundamentally important for combating cancer cells-as well as foreign bodies, such as viruses and bacteria , and combating heart disease and arthritis as well. (newworldencyclopedia.org)
  • Langerhans' Cell Histiocytosis (LCH) is a rare, cancer-like disease that affects cells called Langerhans cells. (scitechdaily.com)
  • LCH, or Langerhans' Cell Histiocytosis, is a serious and potentially fatal disease that primarily affects children. (scitechdaily.com)
  • LCH is a disease in which cancerous mutations occur in immune cells, which typically play a role in detecting and eliminating cancer cells. (scitechdaily.com)
  • Langerhans' Cell Histiocytosis (LCH) is a serious type of cancer-like disease that mainly affects children and can be fatal in severe cases. (sflorg.com)
  • LCH is a disease in which the cancer mutation occurs in the immune cells, which otherwise have the task of detecting and eliminating cancer cells. (sflorg.com)
  • They play a major role in defending the host from both tumors and virally infected cells. (newworldencyclopedia.org)
  • Pancreatic neuroendocrine tumors (also called islet cell tumors) - NETs that typically arise in the pancreas, although they can occur outside the pancreas. (findzebra.com)
  • A culture platform to study quiescent hematopoietic stem cells following genome editing. (u-tokyo-hemat.com)
  • Heterozygous Dnmt3a R878C induces expansion of quiescent hematopoietic stem cell pool. (u-tokyo-hemat.com)
  • This book is about the phenotypic and functional features of epidermal Langerhans cells in experimental models and in clinical medicine. (netlify.app)
  • Available at: http://www.cancernetwork.com/oncology-journal/langerhans-cell-histiocytosis-emerging-insights-and-clinical-implications [Accessed 1 Jul. (capsulehealth.one)
  • Normal histiocyte cells are part of the immune system, alerting infection -fighting cells to the presence of foreign material such as bacteria ( antigen presentation). (dermnetnz.org)
  • The Mononuclear Phagocyte System (MPS) is a group of cells with crucial functions in our immune system: it mediates and regulates inflammation. (cerclefser.org)
  • These cells play a role in the body's immune system. (netlify.app)
  • Histiocyte cells are a form of white blood cells that help the immune system destroy Langerhans cell histiocytosis is a rare disorder that can look like some types of cancer. (netlify.app)
  • B cells are lymphocytes (a class of white blood cells ) that play a large role in the adaptive immune system by making antibodies to identify and neutralize invading pathogens like bacteria and viruses . (newworldencyclopedia.org)
  • Lymphocyte-like natural killer (NK) cells also are involved in the immune system , albeit part of the innate immune system . (newworldencyclopedia.org)
  • Some researchers are convinced that LCH is derived from a certain type of immune cell called dendritic cells, while others believe that they come from related cells called monocytes," says Egle Kvedaraite, a doctor and researcher at the Department of Medical Biochemistry and Biophysics at Karolinska Institutet and first author of the new study. (scitechdaily.com)
  • We also found an IL-17A-dependent pathway for DC fusion, which was highly potentiated by IFN-gamma and led to giant cells expressing three major tissue-destructive enzymes: tartrate resistant acidic phosphatase and matrix metalloproteinases 9 and 12. (dtu.dk)
  • Today we know that DC3 has a separate pathway of development, separate from other dendritic cells and monocytes, and knowledge of this was crucial in our study," says Egle Kvedaraite. (scitechdaily.com)
  • X-rays and PET-CT scans may be performed to see if the affected tissues and cells are present. (homeremedylifestyle.com)
  • Approximately 60% of the patients with LCH carry a mutation in a gene (BRAF) that controls cell growth. (histio.org)
  • It was recently shown that a mutated form of Braf, an oncogene that is involved in directing cell growth, is present in 60% of the patients with LCH. (histio.org)