{"orpha":178540,"name":"Primary cutaneous follicle center lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["PCFCL"],"definition":"A rare, indolent primary cutaneous B-cell lymphoma characterized by a solitary or grouped erythematous plaques or tumors, preferentially located on the head, neck or trunk region, and composed of centroblasts and centrocytes arranged in a follicular, diffuse, or mixed growth pattern. The lesions are smooth and typically do not ulcerate. The neoplastic cells express pan B cell markers and Bcl-6, and typically lack Bcl-2.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-178540/","orphanet_url":"https://www.orpha.net/en/disease/detail/178540","names":{"fr":{"name":"Lymphomes B centrofolliculaire cutané primitif","definition":"Lymphome B cutané primitif d'évolution indolente caractérisé par des plaques ou des tumeurs érythémateuses isolées ou groupées, localisées essentiellement sur la tête, le cou ou le tronc, et composées de centroblastes et de centrocytes avec une croissance folliculaire diffuse ou mixte. Les lésions sont lisses et généralement non ulcéreuses. Les cellules néoplasiques expriment les marqueurs des cellules pan-B et Bcl-6, et sont généralement dépourvues de Bcl-2."},"de":{"name":"Lymphom, primär kutanes, follikuläres","synonyms":["PCFCL"],"definition":"Ein indolentes primäres kutanes B-Zell-Lymphom, das durch solitäre oder gruppierte erythematöse Plaques oder Tumore gekennzeichnet ist, die sich vorzugsweise an Kopf, Hals oder Rumpf befinden und aus Zentroblasten und Zentrozyten bestehen, die in einem follikulären, diffusen oder gemischten Wachstumsmuster angeordnet sind. Die Läsionen sind glatt und weisen in der Regel keine Ulzerationen auf. Die neoplastischen Zellen exprimieren Pan-B-Zell-Marker und Bcl-6, während Bcl-2 typischerweise fehlt."},"es":{"name":"Linfoma cutáneo primario del centro folicular","synonyms":["PCFCL"],"definition":"Es un linfoma cutáneo primario de células B, indolente y poco frecuente, caracterizado por placas eritematosas o tumores aislados o agrupados localizados preferentemente en la región de la cabeza, el cuello o el tronco, y compuestos por centroblastos y centrocitos dispuestos con un patrón de crecimiento folicular, difuso o mixto. Las lesiones son lisas y típicamente no ulcerativas. Las células neoplásicas expresan marcadores panB y Bcl-6, y, por lo general, carecen de Bcl-2."},"it":{"name":"Linfoma cutaneo primitivo del centro follicolare","definition":"È un linfoma cutaneo primitivo silente raro delle cellule B, caratterizzato da tumori o placche eritematose solitarie o raggruppate, localizzate prevalentemente sulla testa, sul collo o sul tronco, composte da centroblasti e centrociti organizzati in una crescita follicolare, diffusa o mista. Le lesioni sono lisce e di solito non si ulcerano. Le cellule neoplastiche esprimono i marcatori cellulari pan B e Bcl-6, mentre di solito mancano i Bcl-2."},"nl":{"name":"Primair cutaan follikelcenterlymfoom","synonyms":["PCFCL"],"definition":"Een zeldzaam, indolent primair cutaan B-cellymfoom gekarakteriseerd door solitaire of gegroepeerde erythemateuze plaques of tumoren, voornamelijk gesitueerd op het hoofd, de nek/hals of de romp, en bestaande uit centroblasten en centrocyten die zijn gerangschikt in een folliculair, diffuus of gemengd groeipatroon. De letsels zijn glad en verzweren doorgaans niet. De neoplastische cellen brengen pan-B-celmerkers en Bcl-6 tot expressie, en missen doorgaans Bcl-2."},"pt":{"name":"Linfoma primário cutâneo do centro folicular"},"pl":{"name":"Pierwotny skórny chłoniak grudkowy","synonyms":["PCFCL"]},"cs":{"name":"Primární kožní B-lymfom z folikulárních center","synonyms":["PCFCL"]},"uk":{"name":"Первинна шкірна лімфома фолікулярного центру","synonyms":["PCFCL"],"definition":"A rare, indolent primary cutaneous B-cell lymphoma characterized by a solitary or grouped erythematous plaques or tumors, preferentially located on the head, neck or trunk region, and composed of centroblasts and centrocytes arranged in a follicular, diffuse, or mixed growth pattern. The lesions are smooth and typically do not ulcerate. The neoplastic cells express pan B cell markers and Bcl-6, and typically lack Bcl-2."},"zh":{"name":"原发性皮肤滤泡中心性淋巴瘤","synonyms":["PCFCL"],"definition":"原发性皮肤滤泡中心淋巴瘤是一种罕见的原发性皮肤惰性B细胞淋巴瘤，其特征是单个或多个的红斑或肿瘤，好发于头部、颈部或躯干，由中心母细胞和中心细胞组成，呈滤泡状、弥漫性或混合生长模式。肿瘤表面光滑，一般不形成溃疡。肿瘤细胞表达B细胞标记物和Bcl-6，不表达Bcl-2。"},"tr":{"name":"Primer kutanöz folikül santral lenfoma","synonyms":["PCFCL"]}},"xrefs":[{"source":"ICD-10","code":"C82.6","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"2A80.3","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"77501812"},{"source":"MONDO","code":"0015814","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1333171","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"13701","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}