{"orpha":86854,"name":"Splenic marginal zone lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["SMZL"],"definition":"Splenic marginal zone lymphoma is a rare, indolent B-cell non-Hodgkin lymphoma characterized by abnormal clonal proliferation of mature B-lymphocytes with involvement in the spleen, bone marrow and, frequently, the blood. It usually presents with splenomegaly, lymphocytosis, anemia and/or thrombocytopenia. Hepatitis C virus and autoimmune manifestations, such as autoimmune hemolytic anemia and autoimmune thrombocytopenia, could be associated.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-86854/","orphanet_url":"https://www.orpha.net/en/disease/detail/86854","names":{"fr":{"name":"Lymphome splénique de la zone marginale","synonyms":["SMZL","LSZM"],"definition":"Le lymphome splénique de la zone marginale est un lymphome rare à lymphocytes B, indolent, non hodgkinien, caractérisé par la prolifération clonale anormale de lymphocytes B matures dans la rate, la moelle osseuse et surtout dans le sang. Il se manifeste habituellement par une splénomégalie, une lymphocytose, une anémie et/ou une thrombocytopénie. Une infection par le virus de l'hépatite C peut être associée, ainsi que des manifestations auto-immunes comme une anémie hémolytique auto-immune ou une thrombocytopénie auto-immune."},"de":{"name":"Splenisches Marginalzonen-Lymphom","synonyms":["SMZL"],"definition":"Das splenisches Marginalzonen-Lymphom ist ein seltenes, indolentes B-Zell-Non-Hodgkin-Lymphom, das durch eine abnorme klonale Proliferation reifer B-Lymphozyten mit Beteiligung der Milz, des Knochenmarks und häufig auch des Blutes gekennzeichnet ist. Es präsentiert sich gewöhnlich mit Splenomegalie, Lymphozytose, Anämie und/oder Thrombozytopenie. Hepatitis-C-Virus und Autoimmunmanifestationen, wie autoimmune hämolytische Anämie und autoimmune Thrombozytopenie, können assoziiert sein."},"es":{"name":"Linfoma esplénico de la zona marginal","synonyms":["SMZL"],"definition":"El linfoma de la zona marginal esplénico es un linfoma no Hodgkin de células B de carácter indolente y poco frecuente caracterizado por una proliferación clonal anómala de linfocitos B maduros con afectación del bazo, la médula ósea y, frecuentemente con expresión en la sangre. Por lo general, se presenta con esplenomegalia, linfocitosis, anemia y/o trombocitopenia. Puede estar asociado al virus de la hepatitis C y a manifestaciones autoinmunes, tales como anemia hemolítica autoinmune y trombocitopenia autoinmune."},"it":{"name":"Linfoma splenico della zona marginale","synonyms":["SMZL"],"definition":"Il linfoma splenico della zona marginale è un linfoma non-Hodgkin a cellule B raro e silente, caratterizzato dalla proliferazione clonale anomala di linfociti B maturi che interessa la milza, il midollo osseo e, spesso, il sangue. Di solito esordisce con splenomegalia, linfocitosi, anemia e/o trombocitopenia. Possono associarsi epatite C e malattie autoimmuni come l'anemia emolitica autoimmune e la trombocitopenia autoimmune."},"nl":{"name":"Splenisch marginale zone lymfoom","synonyms":["SMZL"],"definition":"Splenisch marginale zone lymfoom is een zeldzaam, indolent B-cel non-Hodgkinlymfoom dat wordt gekarakteriseerd door abnormale klonale proliferatie van rijpe B-lymfocyten in de milt, het beenmerg en, frequent, het bloed. De ziekte manifesteert zich meestal met splenomegalie, lymfocytose, anemie en/of trombocytopenie. Hepatitis C-virus en auto-immune symptomen, zoals auto-immune hemolytische anemie en auto-immune trombocytopenie, kunnen geassocieerd zijn."},"pt":{"name":"Linfoma esplénico da zona marginal"},"pl":{"name":"Chłoniak strefy brzeżnej śledziony","synonyms":["SMZL"]},"cs":{"name":"Splenický lymfom z marginální zóny","synonyms":["SMZL"]},"uk":{"name":"Лімфома маргінальної зони селезінки","synonyms":["SMZL"],"definition":"Splenic marginal zone lymphoma is a rare, indolent B-cell non-Hodgkin lymphoma characterized by abnormal clonal proliferation of mature B-lymphocytes with involvement in the spleen, bone marrow and, frequently, the blood. It usually presents with splenomegaly, lymphocytosis, anemia and/or thrombocytopenia. Hepatitis C virus and autoimmune manifestations, such as autoimmune hemolytic anemia and autoimmune thrombocytopenia, could be associated."},"zh":{"name":"脾边缘区淋巴瘤","synonyms":["SMZL"]},"tr":{"name":"Splenik marjinal bölge lenfoması","synonyms":["SMZL"]}},"xrefs":[{"source":"ICD-10","code":"C83.0","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"2A82.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1002343712"},{"source":"ICD-11","code":"XH0MV1","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"904346231"},{"source":"MONDO","code":"0019462","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0349632","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10062113","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":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.5,"area":"Europe","status":"Validated","source":"European Medecines Agency 2013[OTHER]"}],"phenotypes":null}