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  • multisystem
  • A) Somatic mutation of BRAF or other inciting event in CD34 + haematopoietic stem cells or early DC progenitors induces proliferation, maturation and migration of pathological DCs in multiple tissues that results in high-risk multisystem LCH. (nih.gov)
  • chronic
  • Cladribine, sold under the brand name Leustatin among others, is a medication used to treat hairy cell leukemia (HCL, leukemic reticuloendotheliosis) and B-cell chronic lymphocytic leukemia. (wikipedia.org)
  • Cladribine is used for as a first and second-line treatment for symptomatic hairy cell leukemia and for B-cell chronic lymphocytic leukemia and is administered by intravenous infusion. (wikipedia.org)
  • histopathology
  • A high-power magnification view of PLCH histopathology demonstrating infiltration with Langerhans cells (characterized by pale staining cytoplasm and elongated nuclei with delicate folds and clefts and large nucleoli) and scattered eosinophils. (oncologynurseadvisor.com)
  • We report a 1-year-old female infant with isolated Langerhans cell histiocytosis presenting as pruritic papules confined to the vulva, confirmed by histopathology with immunohistochemical staining. (biomedsearch.com)
  • signaling pathway
  • This protein plays a role in regulating the MAP kinase/ERKs signaling pathway, which affects cell division, differentiation, and secretion. (wikipedia.org)
  • inflammatory
  • Histiocytosis X or LCH is a family of related conditions, which are characterized by a distinct inflammatory and proliferative process but which differ from each other regarding what parts of the body are involved. (encyclopedia.com)
  • D) Regardless of cell of origin, the pathological DCs recruit 'bystander' immune cells in an inflammatory lesion characteristic of LCH. (nih.gov)
  • This Langerhans cell-targeted immune escape mechanism seems to be conserved among different HPV genotypes enabling these viruses to remain undetected in the absence of other inflammatory events. (wikipedia.org)
  • Diseases
  • ECD families and patients are also supported by the Histiocytosis Association, Inc. ECD patients, families, and caregivers are encouraged to join the NIH Rare Lung Diseases Consortium Contact Registry. (wikipedia.org)
  • macrophages
  • Phagocytosis is the main process of macrophages and antigen presentation the main property of dendritic cells (so called because of their star-like cytoplasmic processes). (wikipedia.org)
  • syndrome
  • Lymphoma, especially T cell lymphoblastic lymphoma, can present with superior mediastinal syndrome needing urgent empirical therapy without biopsy. (hindawi.com)
  • langerin
  • Langerin is a protein found in Langerhans cells, and other types of dendritic cells. (wikipedia.org)
  • On March 4, 2007 the online Nature Medicine magazine published the research letter "Langerin is a natural barrier to HIV-1 transmission by Langerhans cells. (wikipedia.org)
  • One of the authors of the study, Teunis Geijtenbeek, said that "Langerin is able to scavenge viruses from the surrounding environment, thereby preventing infection" and "since generally all tissues on the outside of our bodies have Langerhans cells, we think that the human body is equipped with an antiviral defense mechanism, destroying incoming viruses. (wikipedia.org)
  • immune
  • Normally, Langerhans cells recognize foreign material, including bacteria, and stimulate the immune system to react to them. (encyclopedia.com)
  • During its natural life cycle, HPV16 infects the basal cells of the epithelium and interacts with Langerhans cells within the epithelial layer, which are responsible for initiating immune responses against epithelial invading pathogens. (wikipedia.org)
  • However, HPV does not activate Langerhans cells in vitro, and this may represent a key mechanism by which HPV evades immune detection in vivo. (wikipedia.org)
  • Specifically, HPV16 entry into Langerhans cells via the annexin A2/S100A10 heterotetramer results in suppressive signaling and lack of Langerhans cell-mediated immune responses. (wikipedia.org)
  • bone
  • They came from the stem cells of the bone marrow , then, they migrate into the bloodstream as monocytes . (biology-online.org)
  • Arenzana-Seisdedos F, Barbey S, Virelizier JL, Kornprobst M, Nezelof C (1986) Histiocytosis X Purified (T6+) cells from bone granuloma produce interleukin 1 and prostaglandin E2 in culture. (springer.com)
  • Coloured Computed Tomography (CT) scan in axial section of the chest of a patient, showing the fracture of a thoracic vertebra (spinal bone T4, green, at lower centre) due to Langerhans cell histiocytosis (LCH). (sciencephoto.com)
  • The derived cells migrate from the bone marrow to the blood as monocytes. (wikipedia.org)
  • infiltration
  • Spontaneous regression is common in these tumors, and it is mediated by infiltration of CD8-expressing T cells followed by expression of Type 1 T helper cell cytokines (such as Interferon-gamma) and recruitment of antitumour effector cells[disambiguation needed]. (wikipedia.org)
  • lymphocytes
  • Cladribine is activated only by lymphocytes, and non-activated cladribine is removed quickly from all other cells. (wikipedia.org)
  • Ernest Beutler and Dennis A. Carson had studied adenosine deaminase deficiency and recognized that because the lack of adenosine deaminase led to the destruction of B cell lymphocytes, a drug designed to inhibit adenosine deaminase might be useful in lymphomas. (wikipedia.org)
  • Benign
  • This, together with the fact that recurrent LAM after lung transplantation carries the same TSC2 mutations as the original LAM, has led to the "benign metastasis" hypothesis that LAM cells can migrate or metastasize from one site to another. (wikipedia.org)
  • In humans, eosinophilic granulomas are considered a benign histiocytosis that occurs mainly in adolescents and young adults. (wikipedia.org)
  • congenital
  • Causes can include: Endogenous sources gonadal tumors (such as arrhenoblastoma), adrenal tumors, germ cell tumor, congenital adrenal hyperplasia, McCune-Albright syndrome, Exogenous hormones Environmental exogenous hormones, As treatment for another condition. (wikipedia.org)
  • tumor
  • DNA damage causes the cell to grow out of control, leading to a tumor . (thetanningguru.com)
  • On a cellular basis, LAM cells carry bi-allelic inactivation of the TSC2 genes, consistent with the "two-hit" tumor suppressor gene model. (wikipedia.org)
  • A variable percentage of cells within the LAM lesion contain mutational inactivation of the Tuberous Sclerosis Complex (TSC1 or TSC2) tumor suppressor genes. (wikipedia.org)
  • Disorders
  • OBJECTIVES: Primary - Determine the impact of the use of umbilical cord blood as a source of hematopoietic stem cells for children with life-threatening oncologic, hematologic, or genetic/metabolic disorders in need of a stem cell transplant. (centerwatch.com)
  • The Mastocytosis Society is a 501(c)3 nonprofit organization dedicated to supporting patients affected by Mastocytosis or Mast Cell Activation Disorders as well as their families, caregivers, and physicians through research, education, and advocacy. (diseaseinfosearch.org)
  • 1. Pesci A, Bertorelli G, Gabrielli M, Olivieri D. Mast cells in fibrotic lung disorders. (ucsf.edu)
  • leukemia
  • It is a driver mutation in a proportion of certain diagnoses, including melanoma, hairy cell leukemia, papillary thyroid carcinoma, colorectal cancer, non-small-cell lung cancer, Langerhans cell histiocytosis, and ameloblastoma. (wikipedia.org)
  • Cladribine, sold under the brand name Leustatin among others, is a medication used to treat hairy cell leukemia (HCL, leukemic reticuloendotheliosis) and B-cell chronic lymphocytic leukemia. (wikipedia.org)
  • Cladribine is used for as a first and second-line treatment for symptomatic hairy cell leukemia and for B-cell chronic lymphocytic leukemia and is administered by intravenous infusion. (wikipedia.org)
  • Carson then synthesized cladribine, and through clinical research at Scripps starting in the 1980s, Beutler tested it as intravenous infusion and found it was especially useful to treat hairy cell leukemia (HCL). (wikipedia.org)
  • neoplastic
  • This often remains true in neoplastic cells from these populations, so that the presence of CD1 antigens can be used in diagnostic immunohistochemistry to identify some thymomas and malignancies arising from T cell precursors. (wikipedia.org)
  • Lung destruction in LAM is a consequence of diffuse infiltration by neoplastic smooth muscle-like cells that invade all lung structures including the lymphatics, airway walls, blood vessels and interstitial spaces. (wikipedia.org)
  • include
  • Its other roles in metabolism include the regulation of glycogen storage, decomposition of red blood cells and the production of hormones. (wikipedia.org)
  • papillary
  • When the tumour cells start to move in a different direction - vertically up into the epidermis and into the papillary dermis - the behaviour of the cells changes dramatically. (thetanningguru.com)
  • human
  • Abcam's MDC Human ELISA (Enzyme-Linked Immunosorbent Assay) kit is an in vitro enzyme-linked immunosorbent assay for the quantitative measurement of Human MDC in serum, plasma, and cell culture supernatants. (abcam.com)
  • CD1 (cluster of differentiation 1) is a family of glycoproteins expressed on the surface of various human antigen-presenting cells. (wikipedia.org)
  • Specific
  • Group 1 CD1 molecules have been shown to present foreign lipid antigens, and specifically a number of mycobacterial cell wall components, to CD1-specific T cells. (wikipedia.org)
  • Treatment
  • They have been reported to be helpful for lichen planus, Grover's disease, Darier's disease, Hailey-Hailey disease and histiocytosis X. Grenz rays have also been used for treatment of actinic keratosis and Bowen's disease. (wikipedia.org)
  • group
  • Soft Tissue Sarcomas (STS) are a heterogeneous group of rare tumors which arise from mesenchymal cells. (clinicsinoncology.com)
  • CD1a, CD1b and CD1c (group 1 CD1 molecules) are expressed on cells specialized for antigen presentation. (wikipedia.org)
  • CD1d (group 2 CD1) is expressed in a wider variety of cells. (wikipedia.org)
  • Group 2 CD1 molecules activate a group of T cells, known as Natural killer T cells because of their expression of NK surface markers such as CD161. (wikipedia.org)
  • stem
  • PURPOSE: This phase II trial is studying how well umbilical cord blood works as a source of stem cells in treating patients with types of cancer as well as other diseases. (centerwatch.com)
  • Compare the incidence of graft-versus-host disease in patients receiving cord blood transplants in this study with historical data for unrelated donor stem cell transplants. (centerwatch.com)
  • natural
  • Natural Killer T (NKT) cells are activated by CD1d-presented antigens, and rapidly produce Th1 and Th2 cytokines, typically represented by interferon-gamma and IL-4 production. (wikipedia.org)
  • However
  • however, it explains the next step in the process of the radial growth, when individual cells start to acquire invasive potential. (thetanningguru.com)