{"orpha":300857,"name":"T-cell/histiocyte rich large B cell lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["THRLBCL"],"definition":"T-cell/histiocyte rich large B cell lymphoma (THRLBCL) is a rare variant of diffuse large B-cell lymphoma (DLBCL), mainly affecting middle-aged men and often not being discovered until an advanced disease stage, with involvement of the spleen, liver and bone marrow occurring at a greater frequency than in DLBCL. It is often difficult to diagnose due to its similarity with other lymphoid diseases such as classic Hodgkin lymphoma and nodular lymphocyte-predominant Hodgkin lymphoma and has an aggressive clinical course.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-300857/","orphanet_url":"https://www.orpha.net/en/disease/detail/300857","names":{"fr":{"name":"Lymphome à grandes cellules B riche en cellules T/histiocytes","synonyms":["THRLBCL"],"definition":"Variante rare du lymphome diffus à grandes cellules&nbsp;B (LDGCB), touchant principalement les hommes d'âge moyen et n'étant souvent détecté qu'à un stade avancé de la maladie, qui touche plus fréquemment la rate, le foie et la moelle osseuse que dans les cas de LDGCB. Il est souvent difficile à diagnostiquer en raison de sa similitude avec d'autres maladies lymphoïdes telles que le lymphome de Hodgkin classique et le lymphome de Hodgkin nodulaire à prédominance lymphocytaire, et son évolution clinique est agressive."},"de":{"name":"T-Zell/histiozytenreiches großzelliges B-Zell-Lymphom","synonyms":["THRLBCL"]},"es":{"name":"Linfoma B de células grandes rico en células -T/histiocitos","synonyms":["THRLBCL"],"definition":"Es una variante poco frecuente del linfoma B difuso de células grandes (DLBCL) que afecta fundamentalmente a varones de mediana edad. Por lo general, no se diagnostica hasta una etapa avanzada de la enfermedad, con afectación del bazo, hígado y médula ósea con mayor frecuencia que en el DLBCL. A menudo, el diagnóstico resulta difícil debido a su similitud con otras enfermedades linfoides, tales como el linfoma de Hodgkin clásico y el linfoma de Hodgkin con predominio de linfocitos nodulares. Tiene un curso clínico agresivo."},"it":{"name":"Linfoma a grandi cellule B ricco in istiociti/cellule T","synonyms":["THRLBCL"],"definition":"Il linfoma a grandi cellule B ricco in istiociti/cellule T (THRLBCL) è una variante rara del linfoma diffuso a grandi cellule B (DLBCL; si veda questo termine), che colpisce soprattutto i maschi di mezza età. Spesso viene diagnosticato solo nello stadio avanzato della malattia, quando è presente un interessamento della milza, del fegato e del midollo osseo, complicanza che si verifica con maggiore frequenza rispetto al DLBCL. Spesso la diagnosi è difficile data la sua somiglianza con altre malattie linfoidi, come il classico linfoma di Hodgkin e il linfoma nodulare di Hodgkin a predominanza linfocitaria (si vedano questi termini). Il decorso clinico è aggressivo."},"nl":{"name":"T-cel/histiocytenrijk grootcellig B-cellymfoom","synonyms":["THRLBCL"],"definition":"T-cel/histiocyt-rijk grootcellig B-cellymfoom (THRLBCL) is een zeldzame variant van diffuus grootcellig B-cellymfoom (DLBCL; zie deze term) dat vooral mannen van middelbare leeftijd treft en vaak niet ontdekt wordt tot in een gevorderd stadium van de ziekte, en waarbij de milt, de lever en het beenmerg frequenter betrokken zijn in vergelijking met DLBCL. Diagnose is vaak moeilijk vanwege de gelijkenis met andere lymfoïde ziekten zoals klassiek Hodgkinlymfoom en nodulair lymfocyt-predominant Hodgkinlymfoom (zie deze termen), en het klinisch verloop is agressief."},"pt":{"name":"Linfoma de células B grandes com abundantes células T / histiócitos"},"pl":{"name":"Chłoniak z dużych komórek B bogaty w komórki T/histiocyty","synonyms":["THRLBCL"]},"cs":{"name":"T-buněčný/na histiocyty bohatý velkobuněčný B-lymfom","synonyms":["THRLBCL"]},"uk":{"name":"Велика В-клітинна лімфома з високим вмістом Т-клітин/гістіоцитів","synonyms":["THRLBCL"],"definition":"T-cell/histiocyte rich large B cell lymphoma (THRLBCL) is a rare variant of diffuse large B-cell lymphoma (DLBCL; see this term), mainly affecting middle-aged men and often not being discovered until an advanced disease stage, with involvement of the spleen, liver and bone marrow occurring at a greater frequency than in DLBCL. It is often difficult to diagnose due to its similarity with other lymphoid diseases such as classic Hodgkin lymphoma and nodular lymphocyte-predominant Hodgkin lymphoma (see these terms) and has an aggressive clinical course."},"zh":{"name":"T细胞组织细胞丰富型大B细胞淋巴瘤","synonyms":["T细胞组织细胞丰富型大B细胞淋巴瘤"],"definition":"富含T淋巴细胞/组织细胞的大B细胞淋巴瘤（THRLBCL）是弥漫性大B细胞淋巴瘤（DLBCL，见本术语）的一种罕见形式，高发于中年男性，常在疾病晚期才被发现，比DLBCL更易累及脾脏、肝脏和骨髓。由于与典型霍奇金淋巴瘤和结节性淋巴细胞为主型霍奇金淋巴瘤（见这些术语）等其他淋巴系统疾病相似，诊断困难且病程长。"},"tr":{"name":"T hücresi / histiositten zengin büyük B hücreli lenfoma","synonyms":["THRLBCL"]}},"xrefs":[{"source":"ICD-10","code":"C83.3","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"2A81.4","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"257833622"},{"source":"ICD-11","code":"XH0WP6","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"2062545636"},{"source":"MONDO","code":"0017597","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1321547","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"12257","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":[],"genes_via_subtypes":[],"inheritance":["Multigenic/multifactorial","Not applicable"],"onset":["Adolescent","Adult","Childhood","Elderly"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.023,"area":"United States","status":"Validated","source":"29845599[PMID]"}],"phenotypes":null}