{"orpha":178544,"name":"Primary cutaneous diffuse large B-cell lymphoma, leg type","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["PCDLBCL,LT"],"definition":"A rare, aggressive, primary cutaneous B-cell lymphoma characterized by rapidly progressive, red to bluish, often ulcerating, nodular tumors predominantly involving the lower legs. Histology shows sheets of centroblasts and immunoblasts that spare the epidermis, but infiltrate the dermis and subcutaneous tissues, and often disseminate extracutaneously. The neoplastic cells typically express CD20, CD79a, Bcl-2, MUM-1, and FOXP1, but are negative for CD10.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-178544/","orphanet_url":"https://www.orpha.net/en/disease/detail/178544","names":{"fr":{"name":"Lymphome cutané à grandes cellules de type jambe","definition":"Lymphome B cutané primitif d'évolution agressive rare, caractérisé par des tumeurs nodulaires rapidement progressives, de couleur rouge à bleuâtre, souvent ulcérées, localisées principalement sur la partie distale des jambes. L'examen histologique montre des nappes de centroblastes et d'immunoblastes épargnant l'épiderme, mais infiltrant le derme et les tissus sous-cutanés, dont la dissémination est souvent extracutanée. Les cellules néoplasiques expriment généralement les CD20, CD79a, Bcl-2, MUM-1 et FOXP1, mais sont négatives pour le CD10."},"de":{"name":"B-Zell-Lymphom, diffuses großzelliges, primär kutanes, vom leg Typ","definition":"Ein seltenes, aggressives, primäres kutanes B-Zell-Lymphom, das durch rasch fortschreitende, rote bis bläuliche, oft ulzerierende, knotige Tumore gekennzeichnet ist, die vorwiegend die Unterschenkel betreffen. Die Histologie zeigt Schichten von Zentroblasten und Immunoblasten, die die Epidermis verschonen, aber die Dermis und das subkutane Gewebe infiltrieren und oft extrakutan streuen. Die neoplastischen Zellen exprimieren typischerweise CD20, CD79a, Bcl-2, MUM-1 und FOXP1, sind aber negativ für CD10."},"es":{"name":"Linfoma difuso cutáneo primario de células B grandes tipo pierna","definition":"Es un linfoma cutáneo primario de células B agresivo y poco frecuente caracterizado por tumores nodulares, a menudo ulcerativos, de tonalidad rojiza a azulada y de progresión rápida, que afectan predominante a la región inferior de las piernas. La histología muestra un infiltrado difuso compuesto por centroblastos e inmunoblastos que respeta la epidermis afectando a la dermis y tejido subcutáneo, con frecuente diseminación extracutánea. Las células neoplásicas, típicamente, expresan CD20, CD79a, Bcl-2, MUM-1 y FOXP1, siendo negativas para CD10."},"it":{"name":"Linfoma cutaneo primitivo diffuso a grandi cellule B, tipo gamba","definition":"È un linfoma cutaneo raro primitivo a cellule B, aggressivo, caratterizzato da tumori nodulari di colore rosso-bluastro, spesso ulcerati, a progressione rapida, localizzati nella porzione inferiore delle gambe. L'esame istologico evidenzia strati di centroblasti ed immunoblasti che risparmiano l'epidermide ma infiltrano il derma e i tessuti sottocutanei, spesso con diffusione extracutanea. Di solito le cellule neoplastiche esprimono CD20, CD79a, Bcl-2, MUM-1 e FOXP1, ma sono negative per CD10."},"nl":{"name":"Primair cutaan diffuus grootcellig B-cellymfoom, been-type","synonyms":["PCDLBCL,LT"],"definition":"Een zeldzaam, agressief, primair cutaan B-cellymfoom, gekarakteriseerd door snel progressieve, rode tot blauwige, vaak etterende, nodulaire tumoren, voornamelijk in de onderbenen. Histologisch onderzoek toont lagen van centroblasten en immunoblasten die de epidermis sparen, maar de dermis en onderhuidse weefsels infiltreren, en vaak buiten de huid verspreiden. De neoplastische cellen brengen doorgaans CD20, CD79a, Bcl-2, MUM-1, en FOXP1 tot expressie, maar zijn negatief voor CD10."},"pt":{"name":"Linfoma primário cutâneo difuso de células B de grande tamanho, tipo perna"},"pl":{"name":"Pierwotny skórny chłoniak rozlany z dużych komórek B, typu nogi","synonyms":["PCDLBCL, LT"]},"cs":{"name":"Primární kožní difúzní velkobuněčný B-lymfom dolních končetin","synonyms":["PCDLBCL,LT"]},"uk":{"name":"Первинна шкірна дифузна велика В-клітинна лімфома, ножний тип","synonyms":["PCDLBCL,LT"],"definition":"A rare, aggressive, primary cutaneous B-cell lymphoma characterized by rapidly progressive, red to bluish, often ulcerating, nodular tumors predominantly involving the lower legs. Histology shows sheets of centroblasts and immunoblasts that spare the epidermis, but infiltrate the dermis and subcutaneous tissues, and often disseminate extracutaneously. The neoplastic cells typically express CD20, CD79a, Bcl-2, MUM-1, and FOXP1, but are negative for CD10."},"zh":{"name":"原发性皮肤弥漫性大B细胞淋巴瘤腿型","synonyms":["PCDLBCL,LT"],"definition":"腿型原发性皮肤弥漫大B细胞淋巴瘤是一种罕见的、侵袭性原发性皮肤B细胞淋巴瘤，其主要临床表现为快速发展的红色或蓝色的溃疡性、结节性肿瘤，主要累及小腿。组织学表现为成片的成中心母细胞和免疫母细胞，不累及表皮，但浸润真皮和皮下组织，并经常向皮肤外扩散。肿瘤细胞通常表达CD20、CD79a、Bcl-2、MUM-1和FOXP1，但不表达CD10。"},"tr":{"name":"Primer kütanöz diffüz büyük B hücreli lenfoma, bacak tipi","synonyms":["PCDLBCL, LT"]}},"xrefs":[{"source":"ICD-10","code":"C83.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2A81.A","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1418101362"},{"source":"MONDO","code":"0006383","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1709656","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":178554,"name":"Aggressive primary cutaneous B-cell lymphoma","classifications":[187,194,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}