{"orpha":86875,"name":"Adult T-cell leukemia/lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["ATLL"],"definition":"A rare, virus associated tumor due to human T-cell leukemia virus type 1 or human T-cell lymphotropic virus type 1 (HTLV-1) and is characterized by the presence of anti-HTLV-1 antibodies, and malignant, mature, medium-sized T cells with condensed chromatin and polylobated nuclei. The malignant cells exhibit a mature CD4+ T cells phenotype and express CD2, CD5, CD25, CD45RO, HLA-DR, and T-cell receptor αβ. Presentation is heterogeneous and is typically of aggressive leukemia or lymphoma, variable skin eruptions, and visceral organ involvement.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-86875/","orphanet_url":"https://www.orpha.net/en/disease/detail/86875","names":{"fr":{"name":"Leucémie/lymphome T de l'adulte","synonyms":["ATLL"],"definition":"Tumeur rare associée au virus T-lymphotrope humain de type 1 (HTLV-1), aussi appelé virus de la leucémie humaine à cellules T de type 1, caractérisée par la présence d'anticorps anti-HTLV-1 et de lymphocytes T malins, matures, de taille moyenne avec chromatine condensée et noyaux polylobés. Les cellules malignes présentent un phénotype de lymphocytes T CD4+ matures et expriment CD2, CD5, CD25, CD45RO, HLA-DR et le récepteur des lymphocytes T de type α/β. La présentation est hétérogène, mais une leucémie ou un lymphome agressif, des éruptions cutanées variables et une atteinte viscérale sont généralement observés."},"de":{"name":"Adulte T-Zell-Leukämie/Lymphom","synonyms":["ATLL"],"definition":"Ein seltener, virusassoziierter Tumor, der durch das humane T-Zell-Leukämievirus Typ 1 oder das humane T-Zell-lymphotrope Virus Typ 1 (HTLV-1) verursacht wird und durch das Vorhandensein von Anti-HTLV-1-Antikörpern und malignen, reifen, mittelgroßen T-Zellen mit kondensiertem Chromatin und polylobierten Kernen gekennzeichnet ist. Die malignen Zellen weisen einen reifen CD4+ T-Zellen-Phänotyp auf und exprimieren CD2, CD5, CD25, CD45RO, HLA-DR und T-Zell-Rezeptor αβ. Das Krankheitsbild ist heterogen und zeigt typischerweise eine aggressive Leukämie oder ein aggressives Lymphom, variable Hauteruptionen und eine Beteiligung viszeraler Organe."},"es":{"name":"Leucemia/linfoma de células T del adulto","synonyms":["ATLL"],"definition":"Es un tumor poco frecuente asociado al virus de la leucemia de células T humanas tipo 1 o virus linfotrópico humano de células T tipo 1 (HTLV-1) y caracterizado por la presencia de anticuerpos anti-HTLV-1 y de células T maduras malignas de tamaño mediano con cromatina condensada y núcleos polilobulados. Las células malignas exhiben un fenotipo de células T CD4 + maduras con expresión de CD2, CD5, CD25, CD45RO, HLA-DR y del receptor αβ de células T. La presentación clínica es heterogénea y consiste típicamente en leucemia o linfoma agresivo, erupciones cutáneas variables y afectación de órganos viscerales."},"it":{"name":"Leucemia/linfoma dell'adulto a cellule T","synonyms":["ATLL"],"definition":"È una neoplasia rara di origine virale, causata dal virus della leucemia a cellule T umana tipo 1 o dal virus linfotropo delle cellule T umane tipo 1 (HTLV-1). È caratterizzata dalla presenza di anticorpi anti-HTLV-1 e di cellule maligne mature di dimensioni medie con cromatina condensata e nuclei polilobati. Le cellule maligne presentano il fenotipo delle cellule T CD4+ mature ed esprimono CD2, CD5, CD25, CD45RO, HLA-DR e il recettore αβ delle cellule T. Il quadro clinico è eterogeneo e caratteristico delle leucemie o dei linfomi aggressivi, con eruzioni cutanee di gravità variabile e interessamento dei visceri."},"nl":{"name":"Volwassen T-celleukemie/lymfoom","synonyms":["ATLL"],"definition":"Een zeldzame, virus-geassocieerde tumor die veroorzaakt wordt door humaan T-cel-leukemie virus type 1 of humaan T-cel-lymfotroop virus type 1 (HTLV-1) en die gekarakteriseerd wordt door de aanwezigheid van anti-HTLV-1-antilichamen, en maligne, rijpe, middelgrote T-cellen met gecondenseerd chromatine en meervoudig gelobde kernen. De maligne cellen vertonen een fenotype van rijpe CD4+ T-cellen en brengen CD2, CD5, CD25, CD45RO, HLA-DR, en T-celreceptor-alfa/bèta tot expressie. De presentatie is heterogeen en omvat doorgaans agressieve leukemie of lymfoom, variabele huiduitslag, en betrokkenheid van inwendige organen."},"pt":{"name":"Leucemia ou linfoma das células T do adulto"},"pl":{"name":"Białaczka/chłoniak dorosłych z komórek T","synonyms":["ATLL"]},"cs":{"name":"Adultní T-buněčný lymfom/leukémie","synonyms":["ATLL"]},"uk":{"name":"Т-клітинна лейкемія/лімфома дорослих","synonyms":["ATLL"],"definition":"A rare, virus associated tumor due to human T-cell leukemia virus type 1 or human T-cell lymphotropic virus type 1 (HTLV-1) and is characterized by the presence of anti-HTLV-1 antibodies, and malignant, mature, medium-sized T cells with condensed chromatin and polylobated nuclei. The malignant cells exhibit a mature CD4+ T cells phenotype and express CD2, CD5, CD25, CD45RO, HLA-DR, and T-cell receptor αβ. Presentation is heterogeneous and is typically of aggressive leukemia or lymphoma, variable skin eruptions, and visceral organ involvement."},"zh":{"name":"成人T细胞白血病/淋巴瘤","synonyms":["ATLL"]},"tr":{"name":"Yetişkin T-hücreli lösemi / lenfoma","synonyms":["ATLL"]}},"xrefs":[{"source":"ICD-10","code":"C91.5","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2A90.5","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"430573082"},{"source":"MONDO","code":"0019471","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D015459","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0023493","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10001413","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]},{"orpha":289635,"name":"Rare virus associated tumor","classifications":[202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Multigenic/multifactorial","Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":3.0,"area":"Europe","status":"Validated","source":"European Medicines Agency 2014[OTHER]"}],"phenotypes":null}