{"orpha":86873,"name":"Aggressive NK-cell leukemia","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["ANKCL","Aggressive NK-cell lymphoma","NK-cell LGL leukemia","NK-cell large granular lymphocyte leukemia"],"definition":"An extremely rare and highly aggressive neoplasm, usually manifesting in the third to fourth decade of life, affecting males and females equally, and characterized by the onset of high fever, weight loss, jaundice, skin infiltration, lymphadenopathy, hepatosplenomegaly, and severe anemia. It has a fulminant and rapidly fatal disease course with the progressive appearance of multiorgan failure and disseminated intravascular coagulation.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-86873/","orphanet_url":"https://www.orpha.net/en/disease/detail/86873","names":{"fr":{"name":"Leucémie agressive à cellules NK","synonyms":["ANKCL","Leucémie à GLG NK","Leucémie à LGL NK","Leucémie à grands lymphocytes granuleux NK"],"definition":"Néoplasie extrêmement rare et très agressive, se manifestant généralement au cours de la troisième ou quatrième décennie de vie, touchant aussi bien les hommes que les femmes. Elle se caractérise par une forte fièvre, une perte de poids, un ictère, une infiltration cutanée, une lymphadénopathie, une hépatosplénomégalie et une anémie sévère. L'évolution de la maladie est fulminante, rapide et létale, avec une défaillance multiviscérale progressive et une coagulation intravasculaire disséminée."},"de":{"name":"Agressive NK-Zell-Leukämie","synonyms":["ANKCL","NK-Zell-LGL-Leukämie","NK-Zell-Leukämie großer granulärer Lymphozyten","NK-Zell-Lymphom, aggressives"]},"es":{"name":"Leucemia agresiva de células NK","synonyms":["ANKCL","Leucemia LGL de células NK","Leucemia linfocítica granular de células NK grandes","Linfoma agresivo de células NK"],"definition":"Es una neoplasia muy poco frecuente y sumamente agresiva que, por lo general, se manifiesta, entre la tercera y cuarta décadas de la vida, afectando por igual a hombres y mujeres. Está caracterizada por la aparición de fiebre alta, pérdida de peso, ictericia, infiltración cutánea, linfadenopatía, hepatoesplenomegalia y anemia grave. Esta leucemia tiene un curso de enfermedad fulminante y rápidamente fatal con la aparición progresiva de fallo multiorgánico y coagulación intravascular diseminada."},"it":{"name":"Leucemia aggressiva a cellule NK","synonyms":["ANKCL","Leucemia a LGL NK","Leucemia a grandi linfociti granulari NK","Linfoma aggressivo a cellule NK"],"definition":"La leucemia aggressiva a cellule NK (ANKCL) è una neoplasia molto rara ed aggressiva, che di solito esordisce nella terza o nella quarta decade di vita e colpisce tanto i maschi quanto le femmine. È caratterizzata da febbre alta, perdita di peso, ittero, infiltrazione della cute, linfoadenopatia, epatosplenomegalia e anemia grave. Il decorso è fulminante, rapido e letale, con insufficienza multiorgano progressiva e coaguli intravascolari diffusi."},"nl":{"name":"Agressieve NK-celleukemie","synonyms":["ANKCL","Agressief NK-cellymfoom","NK-cel LGL-leukemie","NK-cel groot granulair lymfocytleukemie"],"definition":"Een uiterst zeldzaam en zeer agressief neoplasma dat zich meestal manifesteert tijdens het derde tot vierde levensdecennium, dat evenveel mannen als vrouwen treft, en dat gekarakteriseerd wordt door aanvang van hoge koorts, gewichtsverlies, geelzucht, infiltratie van huid, lymfadenopathie, hepatosplenomegalie, en ernstige anemie. Het vertoont een fulminant en snel fataal ziekteverloop met progressief verschijnen van multi-orgaanfalen en verspreide intravasculaire coagulatie."},"pt":{"name":"Leucemia das células NK, agressiva"},"pl":{"name":"Agresywna białaczka z komórek NK","synonyms":["Agresywny chłoniak z komórek NK","ANKCL","Białaczka limfocytarna z dużych ziarnistych komórek NK","NK-cell LGL leukemia"]},"cs":{"name":"Agresivní NK-buněčná leukémie","synonyms":["ANKCL","Agresivní NK-buněčný lymfom","NK-buněčná LGL leukémie","NK-velkobuněčná granulární lymfocytární leukémie"]},"uk":{"name":"Агресивна NK-клітинна лейкемія","synonyms":["Агресивна NK-клітинна лімфома","ANKCL","NK-клітинна велика гранулярна лімфоцитарна лейкемія","NK-клітинна лейкемія LGL"],"definition":"An extremely rare and highly aggressive neoplasm, usually manifesting in the third to fourth decade of life, affecting males and females equally, and characterized by the onset of high fever, weight loss, jaundice, skin infiltration, lymphadenopathy, hepatosplenomegaly, and severe anemia. It has a fulminant and rapidly fatal disease course with the progressive appearance of multiorgan failure and disseminated intravascular coagulation."},"zh":{"name":"侵袭性NK细胞白血病","synonyms":["NK细胞大颗粒淋巴细胞白血病"]},"tr":{"name":"Agresif NK hücreli lösemi","synonyms":["ANKCL","NK hücreli LGL lösemi","Agresif NK hücreli lenfoma","NK hücreli büyük granüler lenfosit lösemi"]}},"xrefs":[{"source":"ICD-10","code":"C94.7","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"2A90.3","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"153957345"},{"source":"MONDO","code":"0019470","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1292777","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10493","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10028811","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":512034,"name":"Large granular lymphocyte leukemia","classifications":[194,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Multigenic/multifactorial","Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Europe","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}