{"orpha":364043,"name":"ALK-positive large B-cell lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["ALK+ LBCL","ALK+ large B-cell lymphoma"],"definition":"A very rare variant of diffuse large B-cell lymphoma (DLBCL) mainly affecting middle-aged immunocompetent men and characterized by a consistent primary involvement of lymph nodes (mainly in the cervical and mediastinum lymph nodes) and with infrequent extra nodal involvement of the bone marrow and other extra-nodal sites (head and neck region, liver, spleen, and gastrointestinal tract). It has an aggressive disease course, and is associated with a poor prognosis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-364043/","orphanet_url":"https://www.orpha.net/en/disease/detail/364043","names":{"fr":{"name":"Lymphome B à grandes cellules ALK positif","synonyms":["ALK+ LBCL","LBGC ALK+","Lymphome B à grandes cellules ALK+"],"definition":"Variante très rare du lymphome B diffus à grandes cellules (LDGCB) affectant principalement les hommes immunocompétents d'âge moyen et caractérisée par une atteinte primaire constante des ganglions lymphatiques (principalement des ganglions lymphatiques cervicaux et médiastinaux) et par une atteinte extra-ganglionnaire peu fréquente de la moelle osseuse et d'autres sites extra-ganglionnaires (région de la tête et du cou, foie, rate et tractus gastro-intestinal). Associée à un pronostic défavorable, la maladie évolue de manière agressive."},"de":{"name":"ALK-positives großzelliges B-Zell-Lymphom","synonyms":["ALK+ LBCL","B-Zell-Lymphom, großzelliges, ALK+"]},"es":{"name":"Linfoma B de células grandes ALK-positivo","synonyms":["ALK+ LBCL","Linfoma B de células grandes ALK+"],"definition":"Es una variante muy poco frecuente de linfoma B difuso B de células grandes (DLBCL) que afecta principalmente a varones inmunocompetentes de mediana edad. Está caracterizado por una afectación primaria constante de los ganglios linfáticos (principalmente de los ganglios cervicales y del mediastino) y con una afectación extranodal infrecuente de la médula ósea y otros sitios extra-nodales (región de cabeza y cuello, hígado, bazo y tracto gastrointestinal). Se asocia a un curso de enfermedad agresivo y a un pronóstico desfavorable."},"it":{"name":"Linfoma a grandi cellule B positivo all'ALK","synonyms":["ALK+ LBCL","Linfoma a grandi cellule B ALK+"],"definition":"Il linfoma diffuso a grandi cellule B positivo alla chinasi del linfoma anaplastico (ALK) è una variante molto rara del linfoma diffuso a grandi cellule B (DLBCL; si veda questo termine), che colpisce soprattutto i maschi immunocompetenti di mezza età. È caratterizzato da un interessamento primario significativo dei linfonodi (soprattutto cervicali e del mediastino), mentre è raro l'interessamento extranodale del midollo osseo e di altri siti (testa e collo, fegato, milza e tratto gastrointestinale). Il decorso della malattia è aggressivo e la prognosi è sfavorevole."},"nl":{"name":"ALK-positief grootcellig B-cellymfoom","synonyms":["ALK+ LBCL","ALK+ grootcellig B-cellymfoom"],"definition":"Een zeer zeldzame variant van diffuus grootcellig B-cellymfoom (DLBCL) die voornamelijk immunocompetente mannen van middelbare leeftijd treft, en die gekarakteriseerd wordt door een consistente primaire betrokkenheid van lymfeklieren (vooral cervicale en mediastinale lymfeklieren) en weinig frequente extranodale betrokkenheid van beenmerg en andere extranodale plaatsen (gebied van hoofd en nek/hals, lever, milt, en maag-darmstelsel). Het vertoont een agressief ziekteverloop en is geassocieerd met een ongunstige prognose."},"pt":{"name":"Linfoma de grandes células BALK - positivo"},"pl":{"name":"Chłoniak ALK-pozytywny z dużych komórek B","synonyms":["ALK+ LBCL","Chłoniak ALK+ z dużych komórek B"]},"cs":{"name":"ALK-pozitivní velkobuněčný B-lymfom","synonyms":["ALK+LBCL","ALK+ velkobuněčný B-lymfom"]},"uk":{"name":"ALK-позитивна велика В-клітинна лімфома","synonyms":["ALK+ велико-В-клітинна лімфома","ALK+ LBCL"],"definition":"A very rare variant of diffuse large B-cell lymphoma (DLBCL) mainly affecting middle-aged immunocompetent men and characterized by a consistent primary involvement of lymph nodes (mainly in the cervical and mediastinum lymph nodes) and with infrequent extra nodal involvement of the bone marrow and other extra-nodal sites (head and neck region, liver, spleen, and gastrointestinal tract). It has an aggressive disease course, and is associated with a poor prognosis."},"zh":{"name":"ALK阳性大B细胞淋巴瘤","synonyms":["ALK+大B细胞淋巴瘤"],"definition":"本病是一种非常罕见的弥漫性大B细胞淋巴瘤(DLBCL)变体，主要影响中年免疫能力正常的男性，其特征是淋巴结(主要在颈部和纵隔淋巴结)的原发性受累一致，骨髓和其他结外部位(头颈部、肝脏、脾脏和胃肠道)的结外受累罕见。本病病程具有侵袭性，并与预后不良相关。"},"tr":{"name":"ALK-pozitif büyük B-hücreli lenfoma","synonyms":["ALK+ LBCL","ALK + büyük B-hücreli lenfoma"]}},"xrefs":[{"source":"ICD-10","code":"C83.3","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"ICD-11","code":"2A81.8","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"2077559619"},{"source":"ICD-11","code":"XH1EB9","qualifier":"BTNT","icd_relation":"specific","validated":true,"icd11_uri":"2047176839"},{"source":"MONDO","code":"0018225","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1333294","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":544,"name":"Diffuse large B-cell lymphoma","classifications":[194,202,233]}],"children":[],"genes":[{"symbol":"ALK","name":"ALK receptor tyrosine kinase","gene_type":"gene with protein product","association":"Part of a fusion gene in","status":"Not yet assessed","source":null,"hgnc":"427","ensembl":"ENSG00000171094","omim":"105590","loci":["2p23.2-p23.1"]}],"genes_via_subtypes":[],"inheritance":["Multigenic/multifactorial","Not applicable"],"onset":[],"prevalence":[],"phenotypes":null}