{"orpha":178536,"name":"Primary cutaneous marginal zone B-cell lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["PCMZL"],"definition":"A rare, indolent primary cutaneous B-cell lymphoma characterized by multifocal, red to violaceous papules, plaques or nodules localized predominantly on the trunk and extremities. Histologically, these are dermis infiltrates consisting of small, marginal zone B cells, lymphoplasmacytic cells, and plasma cells. Marginal zone B cells express CD20, CD79a and Bcl-2, and are negative for CD5, CD10 and Bcl-6. Plasma cells are typically located at the periphery, and express CD138, CD79a, and monotypic light chains.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-178536/","orphanet_url":"https://www.orpha.net/en/disease/detail/178536","names":{"fr":{"name":"Lymphome B cutané de la zone marginale","definition":"Lymphome B cutané primitif d'évolution indolente rare caractérisé par des papules, plaques ou nodules multifocaux, rouges à violacés, localisés principalement sur le tronc et les extrémités. Sur le plan histologique, il s'agit d'un infiltrat dermique composé de petites cellules B de la zone marginale, de lymphoplasmocytes et de plasmocytes. Les cellules B de la zone marginale expriment le CD20, le CD79a et le Bcl-2, et sont négatives pour les CD5, CD10 et Bcl-6. Les cellules plasmatiques sont généralement situées à la périphérie et expriment les CD138, CD79a et des chaînes légères monotypiques."},"de":{"name":"Primär kutanes Marginalzonen-B-Zell-Lymphom","synonyms":["PCMZL"],"definition":"Ein indolentes primäres kutanes B-Zell-Lymphom, das durch multifokale, rote bis violette Papeln, Plaques oder Knötchen gekennzeichnet ist, die vorwiegend am Rumpf und an den Extremitäten auftreten. Histologisch handelt es sich um Dermisinfiltrate, die aus kleinen Marginalzonen-B-Zellen, lymphoplasmatischen Zellen und Plasmazellen bestehen. Marginalzonen-B-Zellen exprimieren CD20, CD79a und Bcl-2 und sind negativ für CD5, CD10 und Bcl-6. Plasmazellen befinden sich typischerweise in der Peripherie und exprimieren CD138, CD79a und monotypische Leichtketten."},"es":{"name":"Linfoma cutáneo primario de células B de la zona marginal","synonyms":["PCMZL"],"definition":"Es un linfoma cutáneo primario de células B poco frecuente e indolente caracterizado por pápulas, placas o nódulos multifocales, de tonalidad rojiza a violácea, localizados predominantemente en el tronco y las extremidades. Histológicamente se observan infiltrados dérmicos que consisten en células B pequeñas de la zona marginal, células linfoplasmocíticas y células plasmáticas. Las células B de la zona marginal expresan CD20, CD79a y Bcl-2, y son negativas para CD5, CD10 y Bcl-6. Las células plasmáticas están típicamente localizadas en la periferia y expresan CD138, CD79a y cadenas ligeras monoclonales."},"it":{"name":"Linfoma cutaneo primitivo della zona marginale a cellule B","definition":"È un linfoma cutaneo primitivo silente raro a cellule B, caratterizzato da papule, placche o noduli multifocali di colore rosso-violaceo, localizzati prevalentemente sul tronco e sugli arti. Gli esami istologici evidenziano infiltrati dermici composti da piccole cellule B della zona marginale, linfoplasmacellule e plasmacellule. Le cellule B della zona marginale esprimono CD20, CD79a e Bcl-2 e sono negative per CD5, CD10 e Bcl-6. Di solito le plasmacellule si localizzano nella zona periferica ed esprimono CD138, CD79a e catene leggere monotipiche."},"nl":{"name":"Primair cutaan marginale zone B-cellymfoom","synonyms":["PCMZL"],"definition":"Een zeldzaam, indolent primair cutaan B-cellymfoom gekarakteriseerd door multifocale, rode tot paarse papels, plaques of noduli, voornamelijk gelokaliseerd op de romp en de ledematen. Histologisch gaat het om infiltraten van de dermis bestaande uit kleine B-cellen uit de marginale zone, lymfoplasmacytaire cellen, en plasmacellen. B-cellen uit de marginale zone brengen CD20, CD79a en Bcl-2 tot expressie, en zijn negatief voor CD5, CD10 en Bcl-6. Plasmacellen zijn doorgaans gelokaliseerd in de periferie, en brengen CD138, CD79a en monotypische lichte ketens tot expressie."},"pt":{"name":"Linfoma primário cutâneo de células B da zona marginal"},"pl":{"name":"Pierwotny skórny chłoniak strefy brzeżnej","synonyms":["PCMZL"]},"cs":{"name":"Primární kožní B-lymfom z marginální zóny","synonyms":["PCMZL"]},"uk":{"name":"Первинна шкірна маргінальна В-клітинна лімфома","synonyms":["PCMZL"],"definition":"A rare, indolent primary cutaneous B-cell lymphoma characterized by multifocal, red to violaceous papules, plaques or nodules localized predominantly on the trunk and extremities. Histologically, these are dermis infiltrates consisting of small, marginal zone B cells, lymphoplasmacytic cells, and plasma cells. Marginal zone B cells express CD20, CD79a and Bcl-2, and are negative for CD5, CD10 and Bcl-6. Plasma cells are typically located at the periphery, and express CD138, CD79a, and monotypic light chains."},"zh":{"name":"原发性皮肤边缘区B细胞淋巴瘤","synonyms":["PCMZL"],"definition":"原发性皮肤边缘区B细胞淋巴瘤是一种罕见的原发性皮肤惰性B细胞淋巴瘤，其主要临床表现为红色或紫色的多发性丘疹、斑块或结节，主要位于躯干和四肢。组织学表现为真皮浸润，由小的边缘区B细胞、淋巴浆细胞和浆细胞组成。边缘区B细胞表达CD20、CD79a和Bcl-2，不表达CD5、CD10或Bcl-6。浆细胞通常位于外周，表达CD138、CD79a和单种轻链。"},"tr":{"name":"Primer kutanöz marjinal bölge B hücreli lenfoma","synonyms":["PCMZL"]}},"xrefs":[{"source":"ICD-10","code":"C83.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2A85.2","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"745285555"},{"source":"MONDO","code":"0015813","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1275321","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":178557,"name":"Indolent primary cutaneous B-cell lymphoma","classifications":[187,194,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}