{"orpha":544,"name":"Diffuse large B-cell lymphoma","type":"Clinical group","group":"Group of disorders","status":"active","flags":[],"synonyms":["DLBCL"],"definition":"Diffuse large B-cell lymphoma is the most common subtype of non-Hodgkin lymphoma (NHL) in adults characterized by a median age of presentation in the sixth decade of life (but also rarely occurring in adolescents and children) with the initial presentation being single or multiple rapidly growing masses (that may or may not be painful) in nodal or extranodal sites (such as thyroid, skin, breast, gastrointestinal tract, testes, bone, or brain) and that can be accompanied by symptoms of fever, night sweats and weight loss. DLBCL has an aggressive disease course, with the elderly having a poorer prognosis than younger patients, and with relapses being common.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-544/","orphanet_url":"https://www.orpha.net/en/disease/detail/544","names":{"fr":{"name":"Lymphome B diffus à grandes cellules","synonyms":["DLBCL","LDGC"],"definition":"Le lymphome B diffus à grandes cellules est le sous-type de lymphome non-hodgkinien le plus fréquent chez l'adulte, avec un âge médian de présentation dans la sixième décennie (plus rarement chez les adolescents et les enfants), se manifestant initialement par une masse unique ou multiples à croissance rapide (douloureuse(s) ou non) de localisation nodale et extra-nodale (thyroïde, peau, sein, tractus gastro-intestinal, testicules, os ou cerveau), et pouvant être accompagnée par une fièvre, des sueurs nocturnes et de perte de poids. Le lymphome B diffus à grandes cellules a une évolution agressive, les patients âgés ayant un plus mauvais pronostic que les patients plus jeunes. Les récidives sont fréquentes."},"de":{"name":"B-Zell-Lymphom, diffuses großzelliges","synonyms":["DLBCL"]},"es":{"name":"Linfoma difuso de células B grandes","synonyms":["LDCBG","DLBCL"],"definition":"Es el subtipo más común de linfoma no Hodgkin (LNH) en adultos y se caracteriza por una mediana de edad de presentación en la sexta década de la vida (pero también ocurre con baja frecuencia en adolescentes y niños) con una presentación inicial de una o varias masas de rápido crecimiento (que pueden o no ser dolorosas) en sitios ganglionares o extraganglionares (como tiroides, piel, mama, tracto digestivo, testículos, hueso o cerebro) y que pueden acompañarse de síntomas como fiebre, sudores nocturnos y pérdida de peso. El LDCGB tiene un curso de enfermedad agresivo. Los adultos de edad avanzada presentan un peor pronóstico que los pacientes más jóvenes, y las recaídas son comunes."},"it":{"name":"Linfoma diffuso a grandi cellule B","synonyms":["DLBCL"]},"nl":{"name":"Diffuus grootcellig B-cellymfoom","synonyms":["DLBCL"],"definition":"Diffuus grootcellig B-cellymfoom (DLBCL) is het meest voorkomende subtype van non-hodgkin-lymfoom (NHL), bij volwassen gekenmerkt door een mediane aanvangsleeftijd in het zesde levensdecennium (maar in zeldzame gevallen ook optredend bij adolescenten en kinderen), en een initiële presentatie met enkel- of meervoudige snelgroeiende massa's (al dan niet pijnlijk) in nodale of extranodale delen (zoals schildklier, huid, borst, maag-darmstelsel, testes, bot, of hersenen), mogelijk vergezeld van symptomen van koorts, nachtzweten en gewichtsverlies. DLBCL kent een agressief ziekteverloop, met een ongunstigere prognose voor oudere dan voor jongere patiënten, en vaak met recidiven."},"pt":{"name":"Linfoma difuso de células B grandes"},"pl":{"name":"Chłoniak rozlany z dużych komórek B","synonyms":["DLBCL"]},"cs":{"name":"Difuzní velkobuněčný B-lymfom","synonyms":["DLBCL"]},"uk":{"name":"Дифузна велика В-клітинна лімфома","synonyms":["DLBCL"],"definition":"Diffuse large B-cell lymphoma is the most common subtype of non-Hodgkin lymphoma (NHL; see this term) in adults characterized by a median age of presentation in the sixth decade of life (but also rarely occurring in adolescents and children) with the initial presentation being single or multiple rapidly growing masses (that may or may not be painful) in nodal or extranodal sites (such as thyroid, skin, breast, gastrointestinal tract, testes, bone, or brain) and that can be accompanied by symptoms of fever, night sweats and weight loss. DLBCL has an aggressive disease course, with the elderly having a poorer prognosis than younger patients, and with relapses being common."},"zh":{"name":"弥漫性大B细胞性淋巴瘤","synonyms":["DLBCL"]},"tr":{"name":"Yaygın büyük B hücreli lenfoma","synonyms":["DLBCL"]}},"xrefs":[{"source":"ICD-10","code":"C83.3","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"2A81","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1946973604"},{"source":"MONDO","code":"0018905","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"18905","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D016403","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0079744","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"3178","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10012818","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":300846,"name":"Aggressive B-cell non-Hodgkin lymphoma","classifications":[194,202,233]}],"children":[{"orpha":364043,"name":"ALK-positive large B-cell lymphoma","type":"Disease"},{"orpha":300849,"name":"Diffuse large B-cell lymphoma of the central nervous system","type":"Disease"},{"orpha":300888,"name":"Diffuse large B-cell lymphoma with chronic inflammation","type":"Disease"},{"orpha":289661,"name":"Epstein-Barr virus-positive diffuse large B-cell lymphoma","type":"Disease"},{"orpha":98839,"name":"Intravascular large B-cell lymphoma","type":"Disease"},{"orpha":86869,"name":"Lymphomatoid granulomatosis","type":"Disease"},{"orpha":98838,"name":"Primary mediastinal large B-cell lymphoma","type":"Disease"},{"orpha":695631,"name":"Primary vitreoretinal large B-cell lymphoma","type":"Disease"},{"orpha":300857,"name":"T-cell/histiocyte rich large B cell lymphoma","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Multigenic/multifactorial","Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-5 / 10 000","value":43.0,"area":"Europe","status":"Validated","source":"European Medicines Agency 2018[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":6.5,"area":"France","status":"Validated","source":"OTHERitut National du Cancer 2013[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.79,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":4.931,"area":"United Kingdom","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":4.773,"area":"Slovenia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":4.78,"area":"Spain","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":6.328,"area":"Switzerland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":6.478,"area":"Netherlands","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.765,"area":"Croatia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.298,"area":"Latvia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.227,"area":"Austria","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":5.51,"area":"Belgium","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":1.229,"area":"Bulgaria","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":3.304,"area":"Czech Republic","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":3.815,"area":"Estonia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":1.244,"area":"Finland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":4.586,"area":"Germany","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":4.559,"area":"Iceland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":3.458,"area":"Ireland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":6.47,"area":"Italy","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.984,"area":"Lithuania","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.827,"area":"Malta","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":5.115,"area":"Norway","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":1.673,"area":"Poland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.635,"area":"Portugal","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":3.259,"area":"Slovakia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}