{"orpha":98841,"name":"Anaplastic large cell lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["ALCL","CD30 positive anaplastic large cell lymphoma","Ki-1 positive anaplastic large cell lymphoma","Primary systemic ALCL","sACL"],"definition":"A rare and aggressive peripheral T-cell non-Hodgkin lymphoma, belonging to the group of CD30-positive lymphoproliferative disorders, which affects lymph nodes and extranodal sites. It is comprised of two sub-types, based on the expression of a protein called anaplastic lymphoma kinase (ALK): ALK positive and ALK negative ALCL.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-98841/","orphanet_url":"https://www.orpha.net/en/disease/detail/98841","names":{"fr":{"name":"Lymphome anaplasique à grandes cellules","synonyms":["ALCL","Lymphome anaplasique à grandes cellules CD30+","Lymphome anaplasique à grandes cellules Ki-1+","Lymphome à grandes cellules anaplasiques"],"definition":"Le lymphome anaplasique à grandes cellules (LAGC) est un lymphome non-Hodgkinien à cellules T périphériques, rare et agressif, appartenant au groupe des syndromes lymphoprolifératifs CD30 positifs et affectant les ganglions lymphatiques et les sites extranodaux. Il comprend deux sous-types, selon l'expression d'une protéine appelée kinase du lymphome anaplasique (ALK) : le LAGC ALK positif et le LAGC ALK négatif."},"de":{"name":"Lymphom, anaplastisch großzelliges","synonyms":["ALCL","CD30-positives anaplastisches großzelliges Lymphom","Ki-1 positives anaplastisches großzelliges Lymphom","sACL"]},"es":{"name":"Linfoma anaplásico de células grandes","synonyms":["ALCL","ALCL sistémico primario","Linfoma anaplásico de células grandes CD30 positivo","Linfoma anaplásico de células grandes Ki-1 positivo","sACL"],"definition":"Es un linfoma periférico no-Hodgkin de células T poco frecuente y agresivo, perteneciente al grupo de los trastornos linfoproliferativos CD30 positivo, que afecta a los ganglios linfáticos y regiones extraganglionares. Según la expresión de una proteína denominada quinasa del linfoma anaplásico (ALK) se clasifica en dos grupos: ALK positivo y ALK negativo."},"it":{"name":"Linfoma anaplastico a grandi cellule","synonyms":["ALCL","ALCL sistemico primitivo","Linfoma anaplastico a grandi cellule CD30-positivo","Linfoma anaplastico a grandi cellule Ki-1-positivo","sACL"],"definition":"Il linfoma anaplastico a grandi cellule (ALCL) è un linfoma periferico non-Hodgkin a cellule T, raro e aggressivo, che appartiene al gruppo delle malattie linfoproliferative CD30-positive e interessa i linfonodi e i siti extranodali. Comprende due sottotipi, basati sull'espressione della proteina ALK (chinasi del linfoma anaplastico): ALCL ALK-positivo e ALK-negativo (si vedano questi termini)."},"nl":{"name":"Anaplastisch grootcellig lymfoom","synonyms":["ALCL","CD30-positief anaplastisch grootcellig lymfoom","Ki-1-positief anaplastisch grootcellig lymfoom","Primair systemisch ALCL","sACL"],"definition":"Een zeldzaam en agressief perifeer T-cel non-Hodgkinlymfoom behorend tot de groep van CD30-positieve lymfoproliferatieve aandoeningen, die de lymfeklieren en locaties buiten de lymfeklieren treffen. Op basis van de expressie van een proteïne genaamd anaplastisch lymfoomkinase (ALK), worden twee subtypes onderscheiden: ALK-positief en ALK-negatief ALCL."},"pt":{"name":"Linfoma anaplásico de células grandes"},"pl":{"name":"Anaplastyczny chłoniak wielkokomórkowy","synonyms":["ALCL","Anaplastyczny chłoniak wielkokomórkowy CD30 pozytywny","Anaplastyczny chłoniak wielkokomórkowy Ki-1 pozytywny","Pierwotny układowy ALCL","sACL"]},"cs":{"name":"Anaplastický velkobuněčný lymfom","synonyms":["ALCL","Primární systémový ALCL","sACL","CD30 pozitivní anaplastický velkobuněčný lymfom","Ki-1 pozitivní anaplastický velkobuněčný lymfom"]},"uk":{"name":"Анапластична великоклітинна лімфома","synonyms":["ALCL","CD30-позитивна анапластична великоклітинна лімфома","Кі-1-позитивна анапластична великоклітинна лімфома","Первинна системна ALCL","sACL"],"definition":"A rare and aggressive peripheral T-cell non-Hodgkin lymphoma, belonging to the group of CD30-positive lymphoproliferative disorders, which affects lymph nodes and extranodal sites. It is comprised of two sub-types, based on the expression of a protein called anaplastic lymphoma kinase (ALK): ALK positive and ALK negative ALCL."},"zh":{"name":"间变性大细胞淋巴瘤","synonyms":["Ki-1阳性间变性大细胞淋巴瘤"],"definition":"该病是一种罕见的侵袭性外周T细胞非霍奇金淋巴瘤，属于CD30阳性淋巴增生性疾病组，累及淋巴结和结外部位。根据间变性淋巴瘤激酶(ALK)蛋白的表达，该病分为两个亚型：ALK阳性和ALK阴性间变性大细胞淋巴瘤。"},"tr":{"name":"Anaplastik büyük hücreli lenfoma","synonyms":["ALCL","sACL","Primer sistemik ALCL","CD30 pozitif anaplastik büyük hücreli lenfoma","Ki-1 pozitif anaplastik büyük hücreli lenfoma"]}},"xrefs":[{"source":"ICD-10","code":"C84.6","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"C84.7","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"2A90.A","qualifier":"BTNT","icd_relation":"specific","validated":true,"icd11_uri":"589245621"},{"source":"ICD-11","code":"2A90.B","qualifier":"BTNT","icd_relation":"specific","validated":true,"icd11_uri":"1179967777"},{"source":"MONDO","code":"0020325","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D017728","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0206180","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"3112","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":171918,"name":"T-cell non-Hodgkin lymphoma","classifications":[194,202,233]}],"children":[{"orpha":300903,"name":"ALK-negative anaplastic large cell lymphoma","type":"Histopathological subtype"},{"orpha":300895,"name":"ALK-positive anaplastic large cell lymphoma","type":"Histopathological subtype"}],"genes":[],"genes_via_subtypes":[{"symbol":"ALK","name":"ALK receptor tyrosine kinase","gene_type":"gene with protein product","association":"Disease-causing somatic mutation(s) in","status":"Assessed","source":"12112524[PMID]","hgnc":"427","ensembl":"ENSG00000171094","omim":"105590","loci":["2p23.2-p23.1"],"via":{"orpha":300895,"name":"ALK-positive anaplastic large cell lymphoma"}}],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":2.0,"area":"Europe","status":"Validated","source":"European Medicines Agency 2011[OTHER]"}],"phenotypes":null}