{"orpha":86872,"name":"T-cell large granular lymphocyte leukemia","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Proliferation of large granular lymphocytes","T-LGL","T-cell LGL leukemia"],"definition":"T-cell large granular lymphocyte leukemia (T-cell LGL leukemia) is a lymphoproliferative malignancy that arises from the mature T-cell (CD3+) lineage.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-86872/","orphanet_url":"https://www.orpha.net/en/disease/detail/86872","names":{"fr":{"name":"Leucémie à grands lymphocytes granuleux T","synonyms":["Leucémie à GLG T","Leucémie à LGL T","Prolifération de grands lymphocytes granuleux","T-GLG","T-LGL"],"definition":"La leucémie à grands lymphocytes granuleux T (leucémie à GLG T) est un cancer lymphoprolifératif qui se développe à partir de la lignée des lymphocytes T matures (CD3+)."},"de":{"name":"T-Zell-Leukämie mit großen granulären Lymphozyten","synonyms":["T-LGL","T-LGL-Leukämie","T-Zell-LGL-Leukämie"],"definition":"Die T-Zell-Leukämie mit großen granulären Lymphozyten (T-LGL-Leukämie) ist eine lymphoproliferative maligne Erkrankung, die aus der reifen T-Zell-Linie (CD3+) hervorgeht."},"es":{"name":"Leucemia linfocítica granular de células T grandes","synonyms":["Leucemia LGL de células T","Proliferación de linfocitos granulares grandes","T-LGL"],"definition":"La leucemia de linfocitos grandes granulares de células T (LLGG de células T) es una neoplasia linfoproliferativa maligna que surge del linaje de células T maduras (CD3+)."},"it":{"name":"Leucemia a grandi linfocitici granulari T","synonyms":["Leucemia LGL a cellule T","Leucemia linfocitica cronica a cellule T","Proliferazione dei grandi linfociti granulari","T-LGL"]},"nl":{"name":"T-celleukemie met grote granulaire lymfocyten","synonyms":["Proliferatie van grote granulaire lymfocyten","T-LGL","T-cel LGL leukemie"],"definition":"T-celleukemie met grote granulaire lymfocyten (T-cel LGL leukemie) is een lymfoproliferatieve maligniteit die ontstaat uit rijpe T-cellen (CD3+)."},"pt":{"name":"Leucemia linfocítica granular grande de células T"},"pl":{"name":"Białaczka z dużych ziarnistych limfocytów T","synonyms":["Proliferacja dużych ziarnistych limfocytów","T-cell LGL białaczka","T-LGL"]},"cs":{"name":"T-velkobuněčná granulární lymfocytární leukémie","synonyms":["T-LGL","Proliferace velkých granulárních lymfocytů","T-buněčná LGL leukémie"]},"uk":{"name":"Т-клітинна велика гранулярна лімфоцитарна лейкемія","synonyms":["Проліферація великих гранулярних лімфоцитів","Т-клітинна лейкемія LGL","T-LGL"],"definition":"T-cell large granular lymphocyte leukemia (T-cell LGL leukemia) is a lymphoproliferative malignancy that arises from the mature T-cell (CD3+) lineage."},"zh":{"name":"T细胞大颗粒淋巴细胞性白血病","synonyms":["大颗粒淋巴细胞增生"]},"tr":{"name":"T-hücreli büyük granüler lenfosit lösemi","synonyms":["T-LGL","T hücreli LGL lösemi","Büyük granüler lenfositlerin çoğalması"]}},"xrefs":[{"source":"ICD-10","code":"C91.7","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"2A90.1","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"83430037"},{"source":"MONDO","code":"0019469","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1955861","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"9812","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10065862","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[{"type":"Moved to","orpha":2687,"name":"Neutropenia-hyperlymphocytosis with large granular lymphocytes syndrome","status":"inactive"}],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":178996,"name":"Acquired neutropenia","classifications":[195]},{"orpha":512034,"name":"Large granular lymphocyte leukemia","classifications":[194,202,233]}],"children":[],"genes":[{"symbol":"STAT3","name":"signal transducer and activator of transcription 3","gene_type":"gene with protein product","association":"Disease-causing somatic mutation(s) in","status":"Assessed","source":"29385279[PMID]_26980727[PMID]","hgnc":"11364","ensembl":"ENSG00000168610","omim":"102582","loci":["17q21.2"]}],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.4,"area":"Europe","status":"Validated","source":"[EXPERT]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}