{"orpha":289661,"name":"Epstein-Barr virus-positive diffuse large B-cell lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["EBV-positive DLBCL","Epstein-Barr virus-positive diffuse large B-cell lymphoma not otherwise specified","Epstein-Barr virus-positive diffuse large B-cell lymphoma, NOS"],"definition":"Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly is a rare form of diffuse large B-cell lymphoma occurring most commonly in patients over the age of 50 (usually between 70-75 years of age), without overt immunodeficiency, and presenting with nodal and extranodal involvement (in sites such as the stomach, lung, skin and pancreas) and B symptoms (fever, night sweats, weight loss). The tumor is characterized by an aggressive course and a short survival rate.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-289661/","orphanet_url":"https://www.orpha.net/en/disease/detail/289661","names":{"fr":{"name":"Lymphome B diffus à grandes cellules positif au virus Epstein-Barr","synonyms":["DLBCL positif à l'EBV","Lymphome B diffus à grandes cellules positif au virus Epstein-Barr, sans autre spécification"],"definition":"Forme rare de lymphome B diffus à grandes cellules qui touche le plus souvent les patients âgés de plus de 50 ans (généralement entre l'âge de 70 et 75 ans), sans immunodéficience manifeste, se caractérisant par une atteinte ganglionnaire et extraganglionnaire (au niveau de l'estomac, des poumons, de la peau et du pancréas, par exemple) et par des symptômes B (fièvre, sueurs nocturnes, perte de poids). La tumeur se caractérise par une évolution agressive et un faible taux de survie."},"de":{"name":"Epstein-Barr Virus-positives diffuses großzelliges B-Zell-Lymphom","synonyms":["EBV-positives DLBCL","Epstein-Barr-Virus-positives diffuses großzelliges B-Zell-Lymphom, nicht weiter spezifiziert"]},"es":{"name":"Linfoma difuso de células B grandes positivo para el virus de Epstein-Barr","synonyms":["LDCBG positivo para EBV","Linfoma difuso de células B grandes positivo para el virus de Epstein-Barr sin otra especificación"],"definition":"Es una forma poco frecuente de linfoma difuso de células B grandes que se presenta con mayor frecuencia en pacientes mayores de 50 años (por lo general, entre los 70 y los 75 años de edad), sin inmunodeficiencia manifiesta, presentando afectación nodal y extranodal (en localizaciones tales como el estómago, los pulmones, la piel y el páncreas) y síntomas B (fiebre, sudores nocturnos, pérdida de peso). El tumor se caracteriza por un curso agresivo y una baja tasa de supervivencia."},"it":{"name":"Linfoma diffuso a grandi cellule B positivo al virus di Epstein-Barr","synonyms":["DLBCL EBV-positivo","Linfoma diffuso a grandi cellule B positivo al virus di Epstein-Barr NAS","Linfoma diffuso a grandi cellule B positivo al virus di Epstein-Barr non altrimenti specificato"],"definition":"Il linfoma diffuso a grandi cellule B positivo al virus di Epstein-Barr dell'età avanzata è una forma rara di linfoma diffuso a grandi cellule B che colpisce soprattutto i pazienti di età superiore a 50 anni (di solito di età compresa tra 70 e 75 anni), in assenza di immunodeficienza conclamata. La malattia esordisce con interessamento nodale ed extranodale (stomaco, polmoni, cute e pancreas) e sintomi correlati ai linfociti B (febbre, sudorazione notturna e perdita di peso). Il decorso è aggressivo e il tasso di sopravvivenza è basso."},"nl":{"name":"Epstein-Barrvirus-positief diffuus grootcellig B-cellymfoom","synonyms":["EBV-positief DLBCL","Epstein-Barrvirus-positief diffuus grootcellig B-cellymfoom niet anderszins omschreven","Epstein-Barrvirus-positief diffuus grootcellig B-cellymfoom, NAO"],"definition":"Epstein-Barrvirus-positief diffuus grootcellig B-cellymfoom bij ouderen is een zeldzame vorm van diffuus grootcellig B-cellymfoom die doorgaans voorkomt bij patiënten ouder dan 50 (meestal tussen 70-75 jaar), zonder zichtbare immuundeficiëntie, en zich manifesteert met nodale en extranodale betrokkenheid (op plaatsen zoals de maag, longen, huid en pancreas) en B-symptomen (koorts, nachtzweten, gewichtsverlies). De tumor wordt gekarakteriseerd door een agressief verloop en een korte overleving."},"pt":{"name":"Linfoma B difuso de grandes células positivo para o vírus Epstein-Barr","synonyms":["DLBCL EBV-positivo","Linfoma B difuso de grandes células positivo para o vírus Epstein-Barr sem outra especificação","Linfoma B difuso de grandes células positivo para o vírus Epstein-Barr, NOS"]},"pl":{"name":"Chłoniak rozlany z dużych komórek B wieku podeszłego wirus Ebsteina-Barra-pozytywny","synonyms":["DLBCL wieku podeszłego EBV-pozytywny","Rozlany chłoniak z komórek B Epstein-Barr dodatni"]},"cs":{"name":"Difuzní velkobuněčný B-lymfom s pozitivitou viru Epsteina-Barrové s nástupem ve vyšším věku","synonyms":["EBV-pozitivní DLBCL ve stáří","Difuzní velkobuněčný B-lymfom s pozitivitou viru Epstein-Barrové s nástupem ve vyšším věku"]},"uk":{"name":"Епштейн-Барр-вірус-позитивна дифузна велико-В-клітинна лімфома","synonyms":["EBV-позитивний DLBCL","Вірусопозитивна дифузна велика В-клітинна лімфома з вірусом Епштейна-Барр, не вказано інше","Вірус-позитивна дифузна велика В-клітинна лімфома Епштейна-Барр, NOS"],"definition":"Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly is a rare form of diffuse large B-cell lymphoma occurring most commonly in patients over the age of 50 (usually between 70-75 years of age), without overt immunodeficiency, and presenting with nodal and extranodal involvement (in sites such as the stomach, lung, skin and pancreas) and B symptoms (fever, night sweats, weight loss). The tumor is characterized by an aggressive course and a short survival rate."},"zh":{"name":"老年人EB病毒阳性弥漫性大B细胞淋巴瘤","synonyms":["老年人EB病毒阳性弥漫性大B细胞淋巴瘤"],"definition":"老年型EB病毒阳性弥漫性大B细胞淋巴瘤是一种罕见的弥漫性大B细胞淋巴瘤，最常见于50岁以上（通常在70-75岁之间）的患者，无明显免疫缺陷，表现为淋巴结和淋巴结外受累（如胃，肺，皮肤和胰腺）和B症状（发热、盗汗、体重减轻）。肿瘤特点为病程呈侵袭性且生存率低。"},"tr":{"name":"Epstein-Barr virüsü-yaşlılarda pozitif yaygın büyük B hücreli lenfoma","synonyms":["Yaşlıların EBV pozitif DLBCL'si"]}},"xrefs":[{"source":"ICD-10","code":"C83.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2A81.6","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"407807101"},{"source":"MONDO","code":"0017346","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2700007","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]},{"orpha":289644,"name":"Epstein-Barr virus-associated malignant lymphoproliferative disorder","classifications":[202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult","Elderly"],"prevalence":[{"type":"Annual incidence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"WHO Classification of Tumours[OTHER]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"WHO Classification of Tumours[OTHER]"}],"phenotypes":null}