• 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)
  • Histiocytosis X was renamed Langerhans-cell histiocytosis , reflecting the concept that LCH cells represented dysfunctional epidermal Langerhans cells. (capsulehealth.one)
  • Available at: http://www.cancernetwork.com/oncology-journal/langerhans-cell-histiocytosis-emerging-insights-and-clinical-implications [Accessed 1 Jul. (capsulehealth.one)
  • C group: Non-Langerhans Cell Histiocytosis (skin). (petmoo.com)
  • L group: Erdheim-Chester Disease, Langerhans Cell Histiocytosis, etc. (petmoo.com)
  • Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the presence of cells with characteristics similar to bone marrow-derived Langerhans cells juxtaposed against a backdrop of hematopoietic cells, including T-cells, macrophages, and eosinophils. (medscape.com)
  • 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)
  • The pathogenesis of Langerhans cell histiocytosis (LCH) is unknown. (medscape.com)
  • Sakata N, Toguchi N, Kimura M, Nakayama M, Kawa K, Takemura T. Development of Langerhans cell histiocytosis associated with chronic active Epstein-Barr virus infection. (mayabouchenaki.com)
  • Murakami I, Gogusev J, Fournet JC, Glorion C, Jaubert F. Detection of molecular cytogenetic aberrations in langerhans cell histiocytosis of bone. (mayabouchenaki.com)
  • Erdem AP, Kasimoglu Y, Sepet E, Gencay K, Sahin S, Dervisoglu S. Oral manifestations may be the first sign of Langerhans cell histiocytosis. (mayabouchenaki.com)
  • Langerhans cell histiocytosis (LCH) is a rare haematological neoplasm characterized by the accumulation of CD1a + , CD207/Langerin + histiocytes within inflammatory lesions. (biomedcentral.com)
  • Langerhans cell histiocytosis (LCH) is an idiopathic group of reactive proliferative diseases linked to aberrant immunity, pathologically characterized by clonal proliferation of Langerhans cells. (engineering.org.cn)
  • In a normal physiological, "non-dangerous" situation, LCs coordinate a continuous state of immune tolerance Langerhans cell histiocytosis (LCH) is a rare disorder in which the body makes too many dendritic cells. (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)
  • 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)
  • Pulmonary Langerhans' cell histiocytosis (PLCH) remains an important diagnostic consideration in the differential diagnosis of diffuse infiltrative lung disease, particular among smokers. (elsevierpure.com)
  • Vassallo, R & Limper, AH 2002, ' Pulmonary Langerhans' cell histiocytosis ', Seminars in Respiratory and Critical Care Medicine , vol. 23, no. 2, pp. 93-101. (elsevierpure.com)
  • Langerhans cell histiocytosis is a rare proliferative disorder of Langerhans cells with uncertain aetiology, wide spectrum of clinical symptoms and varied behaviour. (amedi.sk)
  • Langerhans cell histiocytosis affecting the lungs. (nih.gov)
  • Juvenile xanthogranuloma is a rare non-Langerhans cell histiocytosis that usually occurs during infancy and early childhood. (biomedcentral.com)
  • We present a case of multiorgan, multisite Langerhans cell histiocytosis (LCH), positive for the oncogenic BRAF V600E mutation, in a preterm neonate. (thieme-connect.de)
  • Here, we outline the most likely diagnoses with this presentation and discuss a case of Langerhans cell histiocytosis (LCH) in a preterm neonate with severe multiorgan involvement. (thieme-connect.de)
  • Plasma cell inclusions have been reported occasionally in reactive inflammatory lesions but more frequently in plasma cell tumors and lymphoplasmacytic lymphoma, maybe associated with crystal-storing histiocytosis. (upmc.edu)
  • 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)
  • 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)
  • and reactive, fibrous or vascular lesions including aneurysmal or unicameral bone cysts, fibrous dysplasia, osteomyelitis, Langherhans' cell histiocytosis, non-ossifying fibroma and bone hemangioma. (reviewofophthalmology.com)
  • A 31 year old man was diagnosed in 1986 as having histiocytosis X because of pulmonary fibrosis and radiological maxillary lytic lesions. (bmj.com)
  • Also, for the primary form of immunodeficiency, typical viral hepatitis, infectious cytopenias, reactive arthritis, which are referred to as pseudo-autoimmune lesions. (stop-seizures-meds.com)
  • 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)
  • Histiocytomas lack expression of CD4 and Thy-1, which are consistently expressed by histiocytes in cutaneous and systemic histiocytosis. (wikipedia.org)
  • Reactive angioendotheliomatosis (RAE) is a benign cutaneous vascular disorder characterized by a distinct histologic and clinical appearance. (thedoctorsdoctor.com)
  • Reactive angioendotheliomatosis (RAE) is a rare benign cutaneous vascular proliferation characterized by intravascular hyperplasia of endothelial cells and tuft-like proliferation of vessels. (thedoctorsdoctor.com)
  • We report two patients with reactive cutaneous angioendotheliomatosis appearing distally to arteriovenous fistulas used for hemodialysis because of chronic renal failure. (thedoctorsdoctor.com)
  • Cutaneous histiocytoses are classified according to the type of histiocyte within the skin. (dermnetnz.org)
  • Diffuse dermal angiomatosis: a variant of reactive cutaneous angioendotheliomatosis. (medscape.com)
  • Corti MA, Rongioletti F, Borradori L, Beltraminelli H. Cutaneous reactive angiomatosis with combined histological pattern mimicking a cellulitis. (medscape.com)
  • Malignant histiocytic diseases include malignant histiocytosis (now it is called localized and disseminated histiocytic sarcoma) and cutaneous histiocytoma, which is a benign histiocytic tumor. (petmoo.com)
  • Nonmalignant Nonneoplastic - Reactive (cutaneous or systemic) histiocytosis. (petmoo.com)
  • A number sign (#) is used with this entry because of evidence that histiocytosis and lymphadenopathy with or without cutaneous, cardiac, and/or endocrine features, joint contractures, and/or deafness (histiocytosis-lymphadenopathy plus syndrome) is caused by homozygous or compound heterozygous mutation in the SLC29A3 gene (612373) on chromosome 10q22. (findzebra.com)
  • cell histiocytosis characterized by for evaluation of an asymptomatic skin Complete cutaneous examination re- accumulation of lipid-laden macro- lesion on the penile shaft that has been vealed 10 variably sized CALMs rang- phages. (who.int)
  • Malignant histiocytosis 3.b. (wikipedia.org)
  • M group: Includes Malignant Histiocytosis. (petmoo.com)
  • Treatment protocol for malignant histiocytosis is directed towards controlling the local tumor and tackling the concern for metastasis. (petmoo.com)
  • Malignant histiocytosis cannot be completely cured even with medical management. (petmoo.com)
  • Malignant Histiocytosis is a potentially life-threatening disease in dogs but the mortality rate of dogs is not documented. (petmoo.com)
  • Malignant histiocytosis prognosis for recovery is poor. (petmoo.com)
  • The working group of the Histiocyte Society has divided histocytic disorders into 3 groups: (1) dendritic cell histiocytosis, (2) macrophage-related disorders, and (3) malignant histiocytosis. (medscape.com)
  • Patients with infection-associated HLH usually have persistent unexplained fever, cytopenia, lymphadenopathy, and, frequently, hepatosplenomegaly and coagulopathy, causing diagnostic difficulties with malignant histiocytosis or T-cell lymphoma ( 2 ) . (cdc.gov)
  • 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 for an emerging spectrum of "syndromes" or disorders that involve abnormal proliferation of specialized white blood cells (histiocytes). (petmoo.com)
  • In general, Histiocytosis refers to an abnormally large amount of histiocytes. (petmoo.com)
  • The background shows reactive cellular infiltrates comprising eosinophils, neutrophils, histiocytes, lymphocytes and plasma cells. (radiologycases.my)
  • Hemophagocytic lymphohistiocytosis (HLH) is an uncommon syndrome characterized by a reactive, systemic proliferation of benign histiocytes throughout the reticuloendothelial system ( 1 ) . (cdc.gov)
  • Juvenile xanthogranuloma (JXG) is a benign, self-limiting reactive prolif- erative disorder of non-Langerhans cell histiocytes seen predominantly in infants and young children and occa- sional y in adults. (who.int)
  • Reactive angioendotheliomatosis is a rare benign process that has been mainly described in patients with systemic infections, such as subacute bacterial endocarditis or tuberculosis, and in association with intravascular deposition of cryoproteins. (thedoctorsdoctor.com)
  • Sinus histiocytosis with massive lymphadenopathy (SHML), also designated as Rosai-Dorfman disease (RDD), is a rare benign reactive lymphoproliferative disorder. (bmj.com)
  • It is defined by a characteristic histopathology with sinus histiocytosis and haemophagocytosis known as emperipolesis. (bmj.com)
  • The histiocytosis-lymphadenopathy plus syndrome comprises features of 4 histiocytic disorders previously thought to be distinct: Faisalabad histiocytosis (FHC), sinus histiocytosis with massive lymphadenopathy (SHML), H syndrome, and pigmented hypertrichosis with insulin-dependent diabetes mellitus syndrome (PHID). (findzebra.com)
  • 2005) reported 3 Turkish brothers with sinus histiocytosis and massive lymphadenopathy (SHML). (findzebra.com)
  • General features Non-specific hyperplasia Viral and post-vaccinial HIV infection Mononucleosis Sinus histiocytosis Suppurative lymphadenitis Granulomatous lymphadenitis Toxoplasmosis General features Lymph nodes react to different antigen stimulation with enlargement and expansion of one or all the anatomical areas. (eurocytology.eu)
  • 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)
  • Another rare variant of reactive angioendotheliomatosis has been described in the lower extremities of patients with severe peripheral vascular atherosclerotic disease. (thedoctorsdoctor.com)
  • Kimyai-Asadi A, Nousari HC, Ketabchi N, Henneberry JM, Costarangos C. Diffuse dermal angiomatosis: a variant of reactive angioendotheliomatosis associated with atherosclerosis. (medscape.com)
  • The syndrome was first described in 1939 as poorly-controlled histiocyte proliferation, but has since also been called hemophagocytic histiocytosis and macrophage activation syndrome [ 5 - 7 ]. (biomedcentral.com)
  • Reactive histiocytosis (immunohistochemical features show that interstitial/dermal DCs are involved) 2.a. (wikipedia.org)
  • Intravascular and diffuse dermal reactive angioendotheliomatosis secondary to iatrogenic arteriovenous fistulas. (thedoctorsdoctor.com)
  • This second variant has been named diffuse dermal reactive angioendotheliomatosis. (thedoctorsdoctor.com)
  • The first patient showed intravascular reactive angioendotheliomatosis, while the second one had purpuric plaques that were characterized histopathologically by diffuse dermal angioendotheliomatosis. (thedoctorsdoctor.com)
  • Requena L, FariƱa MC, Renedo G, Alvarez A, Yus ES, Sangueza OP. Intravascular and diffuse dermal reactive angioendotheliomatosis secondary to iatrogenic arteriovenous fistulas. (medscape.com)
  • Reactive angioendotheliomatosis is associated with several disorders, including infectious diseases (especially subacute endocarditis), antiphospholipid syndrome, dysglobulinemia, and cryoproteinemia. (thedoctorsdoctor.com)
  • Creamer D, Black MM, Calonje E. Reactive angioendotheliomatosis in association with the antiphospholipid syndrome. (medscape.com)
  • Kawaoka J, McBean J, Li H, Mihm MC Jr, Kroumpouzos G. Coexistence of diffuse reactive angioendotheliomatosis and neutrophilic dermatosis heralding primary antiphospholipid syndrome. (medscape.com)
  • Reactive angioendotheliomatosis in a patient with Myelodysplastic Syndrome presenting as a cellulitis-like plaque. (medscape.com)
  • We describe the first case of hemorrhagic fever with renal syndrome due to Hantaan virus presenting with reactive hemophagocytosis. (cdc.gov)
  • HS is often described in binary terms as "primary," indicating Mendelian inheritance of gene mutations resulting in cytotoxic lymphocyte dys- function, or "secondary" indicating an acquired reactive disorder. (scirp.org)
  • On the contrary, LCH is a reactive disorder with underlying neoplastic potential. (mayabouchenaki.com)
  • Recent progress in the clinical approach to these patients has emphasized important roles for high-resolution computed tomographic (CT) scanning and immune reactive tissue markers including cluster differentiation (CD) 1a antigen in the diagnosis of this disorder. (elsevierpure.com)
  • The pathogenesis is unknown, although the disease is believed to be a reactive rather than a neoplastic process. (biomedcentral.com)
  • The pathogenesis of JXG is thought to be of reactive origin, namely local tissue injury that evoked a histioxanthomatous reaction. (eyewiki.org)
  • It may be possible to tell which type of histiocytosis is present by the appearance of the skin lesion or rash , but a skin biopsy is usually necessary. (dermnetnz.org)
  • Susan We do kappa/lambda on trephine marrows using Dako envision and proteinase -k also.The predilute works well for both, diluted 1/2 using reactive lymph node as control. (utsouthwestern.edu)
  • Histiocytic diseases in dogs are a group of diseases in dogs which may involve the skin, and which can be difficult to differentiate from granulomatous, reactive inflammatory or lymphoproliferative diseases. (wikipedia.org)
  • JXG does not carry BRAF V600E mutation, except in patients with both Langerhans' cells histiocytosis (LCH) and JXG (non-Langerhan cells histiocytosis) diseases and in the aggressive forms of JXG. (eyewiki.org)
  • It has been debated whether LCH is a reactive or neoplastic process. (medscape.com)
  • Primary and secondary intralymphatic histiocytosis. (medscape.com)
  • It can be divided into a primary genetic form and a secondary reactive form (Table 1 ), a distinction that has historically been used to differentiate cases of often fatal infantile HPS from those caused by other etiologies that appear later in life and have a better prognosis. (biomedcentral.com)
  • It was considered to be specific bone involvement in histiocytosis X. Chemotherapy (six regimens of vinblastin and corticosteroids, then two regimens of methotrexate) was administered. (bmj.com)
  • 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)
  • A histiocytosis is a disease in which there are too many histiocyte cells in the skin and other organs. (dermnetnz.org)
  • [ 2 ] The arachnoid cells have several proposed functions, including acting as a structural barrier with cellular wrapping/ensheathing, acting as a conduit for cerebrospinal fluid (CSF) drainage/absorption into dural sinuses/veins (arachnoid villi), epithelial-like/secretory functions, monocytelike functions, trophic support and byproduct detoxification for glial and neuronal cells, and participation in reactive/reparative processes. (medscape.com)