{"orpha":178522,"name":"Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare, primary cutaneous T-cell lymphoma characterized by solitary cutaneous nodule or only regional disease, typically occurring on the head and neck, and involving entire dermis. Sometimes, subcutis and adnexal structures are involved, as well. The infiltrate is nodular or diffuse, composed of small to medium sized pleomorphic lymphocytes and showing mild to moderate cytologic atypia. Neoplastic T-cells are mixed with B-cells, histiocytes, plasma cells and eosinophils.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-178522/","orphanet_url":"https://www.orpha.net/en/disease/detail/178522","names":{"fr":{"name":"Lymphome T cutané à cellules pléomorphes de taille petite à moyenne CD4+","definition":"Lymphome T cutané primitif rare, caractérisé par un nodule cutané isolé ou une atteinte uniquement circonscrite, survenant généralement au niveau de la tête et du cou et touchant l'ensemble du derme. Parfois, les structures sous-cutanées et annexielles sont également touchées. L'infiltrat est nodulaire ou diffus, composé de lymphocytes pléomorphes de taille petite à moyenne, présentant des atypies cytologiques légères à modérées. Les lymphocytes T néoplasiques sont mélangés à des lymphocytes B, des histiocytes, des plasmocytes et des éosinophiles."},"de":{"name":"T-Zell-Lymphom, primär kutanes, klein/mittelgroßzelliges pleomorphes, CD4-+"},"es":{"name":"Linfoma cutáneo primario pleomórfico de células T CD4+ pleomórficas de pequeño y mediano tamaño","definition":"Es un linfoma cutáneo primario de células T, poco frecuente, caracterizado por un nódulo cutáneo solitario o enfermedad regional únicamente, que suele aparecer en la cabeza y el cuello, y que afecta a toda la dermis. En ocasiones, el subcutis y las estructuras anexas también se ven afectadas. El infiltrado es nodular o difuso, compuesto por linfocitos pleomórficos de tamaño pequeño a mediano y con atipia citológica de leve a moderada. Las células T neoplásicas están mezcladas con células B, histiocitos, células plasmáticas y eosinófilos."},"it":{"name":"Linfoma cutaneo pleomorfo primitivo a cellule T CD4+ di piccole-medie dimensioni","definition":"Si tratta di un linfoma cutaneo primitivo a cellule T raro, caratterizzato da un nodulo cutaneo solitario o da una localizzazione esclusivamente regionale, che di solito si manifesta sulla testa e sul collo, ed interessa tutto il derma. Occasionalmente la malattia interessa anche la regione sottocutanea e gli annessi cutanei. L'infiltrato è nodulare o diffuso, è composto da linfociti pleomorfi di piccole-medie dimensioni, con atipie citologiche lievi-moderate. Le cellule T neoplastiche sono frammiste alle cellule B, agli istiociti, alle plasmacellule e agli eosinofili."},"nl":{"name":"Primair cutaan CD4+ klein/middelgroot pleomorf T-cellymfoom","synonyms":["Primair cutaan CD4+ klein/middelgroot pleiomorf T-cellymfoom"],"definition":"Een zeldzaam primair cutaan T-cellymfoom, gekenmerkt door een solitaire cutane nodulus of enkel lokale ziekte, doorgaans aan hoofd en hals, en met betrokkenheid van gehele dermis. Soms zijn ook subcutis en adnexen betrokken. Het infiltraat is nodulair of diffuus, bestaande uit kleine tot middelgrote pleomorfe lymfocyten, en met milde tot matige cytologische atypie. Neoplastische T-cellen zijn gemengd met B-cellen, histiocyten, plasmacellen en eosinofielen."},"pt":{"name":"Linfoma primário cutâneo de células T CD4+ pleomórficas e de pequeno/médio tamanho"},"pl":{"name":"Pierwotny chłoniak skóry z małych/średnich pleomorficznych komórek T CD4+"},"cs":{"name":"Primární kožní lymfom s CD4+ malými/středně velkými pleomorfními T-buňkami"},"uk":{"name":"Первинна шкірна CD4+ плеоморфних Т-клітин малих/середніх розмірів лімфома","definition":"A rare, primary cutaneous T-cell lymphoma characterized by solitary cutaneous nodule or only regional disease, typically occurring on the head and neck, and involving entire dermis. Sometimes, subcutis and adnexal structures are involved, as well. The infiltrate is nodular or diffuse, composed of small to medium sized pleomorphic lymphocytes and showing mild to moderate cytologic atypia. Neoplastic T-cells are mixed with B-cells, histiocytes, plasma cells and eosinophils."},"zh":{"name":"原发性皮肤CD4+多形性小/中T细胞淋巴瘤","definition":"原发性皮肤小/中型多形性CD4+T淋巴瘤是一种罕见的原发性皮肤T细胞淋巴瘤，其主要临床表现为孤立性皮肤结节或局部病变，通常发生在头颈部，累及整个真皮，有时累及皮下组织和附件结构。大小不等的多形性淋巴细胞构成结节状或弥漫性浸润物，表现出轻度到中度的细胞异型性。T淋巴细胞与B淋巴细胞、组织细胞、浆细胞和嗜酸性粒细胞混合。"},"tr":{"name":"Primer kutanöz CD4 + küçük / orta boy pleomorfik T hücreli lenfoma"}},"xrefs":[{"source":"ICD-10","code":"C84.4","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2B0Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1278180128"},{"source":"ICD-11","code":"XH7EL2","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0015810","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5680503","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":178548,"name":"Indolent primary cutaneous T-cell lymphoma","classifications":[187,194,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}