{"orpha":86885,"name":"Primary cutaneous peripheral T-cell lymphoma not otherwise specified","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Primary cutaneous peripheral T-cell lymphoma NOS","Primary cutaneous unspecified peripheral T-cell lymphoma"],"definition":"An extremely rare, primary cutaneous T-cell lymphoma disorder characterized by solitary, or multifocal and diffuse, cutaneous lesions, ranging from tumor-like patches, plaques, papules, nodules, and/or erythroderma, located on any area of the body, which rapidly progress and may become ulcerated and/or infected. Systemic involvement may be associated.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-86885/","orphanet_url":"https://www.orpha.net/en/disease/detail/86885","names":{"fr":{"name":"Lymphome cutané primitif à cellules T périphérique sans autre indication","synonyms":["Lymphome cutané primitif à cellules T périphérique SAI","Lymphome cutané primitif à cellules T périphérique non spécifié"],"definition":"Lymphome T cutané primitif rare caractérisé par des lésions cutanées solitaires ou multifocales et diffuses, à type de taches semblables à des tumeurs, de plaques, de papules, de nodules et/ou d'érythrodermie, pouvant siéger sur toute partie du corps. De progression rapide, ces lésions peuvent s'ulcérer et/ou s'infecter. Une atteinte systémique peut être associée."},"de":{"name":"Primär kutanes peripheres T-Zell-Lymphom, andernorts nicht klassifiziert","synonyms":["PTCL-NOS","Primär kutanes PTCL ohne weitere Spezifizierung","T-Zell-Lymphom, peripheres, primär kutanes, unspezifisches"]},"es":{"name":"Linfoma cutáneo primario de células T periféricas sin otra especificación","synonyms":["Linfoma cutáneo primario de células T periféricas inespecífico"],"definition":"Es un linfoma cutáneo primario de células T extremadamente infrecuente caracterizado por lesiones cutáneas aisladas o multifocales y difusas, tales como manchas de tipo tumoral, placas, pápulas, nódulos y/o eritrodermia que se localizan en cualquier parte del cuerpo, de progresión rápida y que pueden llegar a ulcerarse y/o infectarse. Este trastorno puede asociar afectación sistémica."},"it":{"name":"Linfoma cutaneo periferico aspecifico primitivo a cellule T","definition":"È un linfoma cutaneo primitivo a cellule T molto raro, caratterizzato da una lesione cutanea isolata o da lesioni multifocali diffuse (che comprendono le chiazze simil-tumorali, le placche, le papule, i noduli e/o l'eritrodermia), localizzate in qualsiasi zona del corpo, che progrediscono rapidamente, si ulcerano e/o si infettano. È possibile un interessamento sistemico."},"nl":{"name":"Primair cutaan perifeer T-cellymfoom niet anders gespecificeerd","synonyms":["Primair cutaan niet-gespecificeerd perifeer T-cellymfoom","Primair cutaan perifeer T-cellymfoom NOS"],"definition":"Een uiterst zeldzaam, primair, cutaan T-cellymfoom gekarakteriseerd door solitaire of multifocale en diffuse, cutane laesies, gaande van tumor-achtige plekken, plaques, papels, noduli, en/of erytrodermie, die zich situeren op eender welk gebied van het lichaam, die een snelle progressie kennen, en die kunnen geülcereerd en/of geïnfecteerd raken. Systemische betrokkenheid kan geassocieerd zijn."},"pt":{"name":"Linfoma de células T periféricas, inespecífico"},"pl":{"name":"Pierwotny chłoniak skórny z obwodowych komórek T, bliżej nieokreślony","synonyms":["Pierwotny skórny chłoniak z obwodowych komórek T NOS"]},"cs":{"name":"Primární kožní nespecifikovaný periferní T-buněčný lymfom","synonyms":["Primary cutaneous peripheral T-cell lymphoma NOS","Primary cutaneous unspecified peripheral T-cell lymphoma"]},"uk":{"name":"Первинна шкірна периферична Т-клітинна лімфома, не спеціфікована","synonyms":["Первинна шкірна периферична Т-клітинна лімфома NOS","Первинна шкірна неспецифічна периферична Т-клітинна лімфома"],"definition":"An extremely rare, primary cutaneous T-cell lymphoma disorder characterized by solitary, or multifocal and diffuse, cutaneous lesions, ranging from tumor-like patches, plaques, papules, nodules, and/or erythroderma, located on any area of the body, which rapidly progress and may become ulcerated and/or infected. Systemic involvement may be associated."},"zh":{"name":"原发性皮肤外周T细胞淋巴瘤，未分类","synonyms":["原发性皮肤未分类外周T淋巴细胞白血病"]},"tr":{"name":"Primer kutanöz periferik T hücreli lenfoma, başka türlü tanımlanmamış","synonyms":["Primer kutanöz periferik T hücreli lenfoma NOS","Primer kutanöz tanımlanmamış periferik T hücreli lenfoma"]}},"xrefs":[{"source":"ICD-10","code":"C84.4","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2A90.C","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1998421548"},{"source":"MONDO","code":"0019476","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D016411","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0079774","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10034623","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":178551,"name":"Aggressive primary cutaneous T-cell lymphoma","classifications":[187,194,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult","Elderly"],"prevalence":[],"phenotypes":null}