{"orpha":86867,"name":"Nodal marginal zone B-cell lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["NMZL"],"definition":"Nodal marginal zone B-cell lymphoma is a rare, indolent B-cell non-Hodgkin lymphoma, characterized by abnormal clonal proliferation of mature B-lymphocytes with involvement of the lymph nodes, sometimes the bone marrow, and rarely the blood. Clinically it presents with disseminated peripheral, abdominal and/or thoracic lymphadenopathy. Cytopenia and bulky tumors (greater than 5 cm) are rare. Association with Hepatitis C virus and chronic inflammation has been reported.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-86867/","orphanet_url":"https://www.orpha.net/en/disease/detail/86867","names":{"fr":{"name":"Lymphome nodulaire à cellules B de la zone marginale","synonyms":["LNZM","NMZL"],"definition":"Le lymphome nodulaire à cellules B de la zone marginale est un lymphome non Hodgkinien à cellules B, indolent et rare, caractérisé par la prolifération clonale anormale de lymphocytes B matures, avec atteinte des ganglions lymphatiques, parfois de la moelle osseuse, et dans de rares cas du sang. Sur le plan clinique, il se manifeste par une lymphadénopathie périphérique, abdominale et/ou thoracique disséminée. La cytopénie et les tumeurs volumineuses (plus de 5 cm) sont rares. Une association avec le virus de l'hépatite C et une inflammation chronique a été rapportée."},"de":{"name":"Nodales Marginalzonen-B-Zell-Lymphom","synonyms":["NMZL"],"definition":"Das nodale Marginalzonen-B-Zell-Lymphom ist ein seltenes, indolentes B-Zell-Non-Hodgkin-Lymphom, das durch abnorme klonale Proliferation reifer B-Lymphozyten mit Beteiligung der Lymphknoten, manchmal des Knochenmarks und selten des Blutes gekennzeichnet ist. Klinisch präsentiert es sich mit disseminierter peripherer, abdominaler und/oder thorakaler Lymphadenopathie. Zytopenie und voluminöse Tumore (größer als 5 cm) sind selten. Es wurde über eine Assoziation mit dem Hepatitis-C-Virus und chronischen Entzündungen berichtet."},"es":{"name":"Linfoma nodal de zona marginal de células B","synonyms":["NMZL"],"definition":"El linfoma nodal de zona marginal de células B es un linfoma no-Hodgkin de células B, indolente y poco frecuente, caracterizado por una proliferación clonal anómala de linfocitos B maduros con afectación de los nódulos linfáticos, en ocasiones de la médula ósea y, raras veces, de la sangre. Clínicamente se presenta con linfadenopatía periférica, abdominal y/o torácica diseminada. La citopenia y los tumores voluminosos (mayores de 5 cm) son infrecuentes. Se ha descrito una asociación con el virus de la Hepatitis C e inflamación crónica."},"it":{"name":"Linfoma nodulare a cellule B della zona marginale","synonyms":["NMZL"],"definition":"Il linfoma nodulare a cellule B della zona marginale è un linfoma non-Hodgkin, a cellule B, raro e indolente, caratterizzato da un'anomala proliferazione clonale di linfociti B maturi con interessamento dei linfonodi, occasionalmente del midollo osseo e raramente del sangue. Esordisce con una linfadenopatia disseminata periferica, addominale e/o toracica. Sono rari la citopenia e i tumori di dimensioni superiori a 5 cm. Sono stati descritti pazienti con epatite C e infiammazione cronica."},"nl":{"name":"Nodaal marginale zone B-cellymfoom","synonyms":["NMZL"],"definition":"Nodaal marginale zone B-cellymfoom is een zeldzaam, indolent non-Hodgkinlymfoom dat gekarakteriseerd wordt door abnormale klonale proliferatie van rijpe B-lymfocyten met betrokkenheid van de lymfeklieren, soms het beenmerg, en zelden het bloed. Klinisch vertoont de ziekte zich met verspreide perifere, abdominale en/of thoracale lymfadenopathie. Cytopenie en omvangrijke tumoren (groter dan 5 cm) komen zelden voor. Associatie met hepatitis C-virus en chronische ontsteking werd reeds gerapporteerd."},"pt":{"name":"Linfoma nodal de células B da zona marginal"},"pl":{"name":"Węzłowy chłoniak strefy brzeżnej B-komórkowy","synonyms":["NMZL"]},"cs":{"name":"Uzlinový B-buněčný lymfom z marginální zóny","synonyms":["NMZL"]},"uk":{"name":"Вузлова маргінальна В-клітинна лімфома","synonyms":["NMZL"],"definition":"Nodal marginal zone B-cell lymphoma is a rare, indolent B-cell non-Hodgkin lymphoma, characterized by abnormal clonal proliferation of mature B-lymphocytes with involvement of the lymph nodes, sometimes the bone marrow, and rarely the blood. Clinically it presents with disseminated peripheral, abdominal and/or thoracic lymphadenopathy. Cytopenia and bulky tumors (greater than 5 cm) are rare. Association with Hepatitis C virus and chronic inflammation has been reported."},"zh":{"name":"淋巴结边缘区B细胞淋巴瘤","synonyms":["NMZL"]},"tr":{"name":"Nodal marjinal bölge B-hücreli lenfoma","synonyms":["NMZL"]}},"xrefs":[{"source":"ICD-10","code":"C83.0","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"2A85.0","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1720785883"},{"source":"MONDO","code":"0019465","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0855139","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10029460","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":300912,"name":"Marginal zone lymphoma","classifications":[194,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adolescent","Adult","Elderly"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":1.0,"area":"Europe","status":"Validated","source":"European Medecines Agency 2013[OTHER]"}],"phenotypes":null}