{"orpha":48686,"name":"Primary effusion lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Body cavity-based lymphoma","PEL"],"definition":"Primary effusion lymphoma (PEL) is a large B-cell lymphoma located in the body cavities, characterized by pleural, peritoneal, and pericardial fluid lymphomatous effusions and that is always associated with human herpes virus-8 (HHV-8).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-48686/","orphanet_url":"https://www.orpha.net/en/disease/detail/48686","names":{"fr":{"name":"Lymphome primitif des séreuses","synonyms":["PEL","Lymphome des cavités"],"definition":"Le lymphome primitif des séreuses (LPS) est un lymphome à grandes cellules B localisé dans les cavités corporelles, caractérisé par des épanchements de fluide pleural, péritonéal et péricardiaque, et toujours associés à l'herpès-virus humain 8 (HHV-8)."},"de":{"name":"Primäres Effusionslymphom","synonyms":["PEL"],"definition":"Das primäre Effusions-Lymphom (PEL) ist ein in den Körperhöhlen lokalisiertes großzelliges B-Zell-Lymphom und gekennzeichnet durch lymphomatöse Pleura-, Peritoneum- und Perikardergüsse. Immer ist es mit dem humanen Herpes-Virus 8 (HHV-8) assoziiert."},"es":{"name":"Linfoma primario de efusiones","synonyms":["Linfoma primario de cavidades","PEL"],"definition":"El linfoma primario de cavidades (LEP) es un linfoma de células B grandes localizado en las cavidades corporales, caracterizado por derrame linfomatoso pleural, peritoneal y pericárdico, y siempre está asociado con el virus herpes humano 8 (HHV-8)."},"it":{"name":"Linfoma primitivo effusivo","synonyms":["Linfoma delle cavità corporee","PEL"],"definition":"Il linfoma primitivo effusivo (PEL) è un linfoma a grandi cellule B, localizzato nelle cavità del corpo; è caratterizzato da versamenti linfomatosi pleurici, peritoneali e pericardici, in associazione con l'infezione da virus herpetico umano tipo 8 (HHV-8)."},"nl":{"name":"Primair effusielymfoom","synonyms":["Op lichaamsholte gebaseerd lymfoom","PEL"],"definition":"Primair effusielymfoom (PEL) is een grootcellig B-cellymfoom gesitueerd in lichaamsholtes, gekenmerkt door lymfomateuze effusies van pleuraal, peritoneaal en pericardiaal vocht en steeds geassocieerd met humaan herpesvirus-8 (HHV-8)."},"pt":{"name":"Linfoma de derrame primário","synonyms":["PEL"]},"pl":{"name":"Pierwotny chłoniak wysiękowy","synonyms":["PEL"]},"cs":{"name":"Primární efuzní lymfom","synonyms":["PEL","Lymfom v tělních dutinách"]},"uk":{"name":"Первинна випотна лімфома","synonyms":["Лімфома на основі порожнин тіла","PEL"],"definition":"Primary effusion lymphoma (PEL) is a large B-cell lymphoma located in the body cavities, characterized by pleural, peritoneal, and pericardial fluid lymphomatous effusions and that is always associated with human herpes virus-8 (HHV-8)."},"zh":{"name":"原发渗出性淋巴瘤","synonyms":["原发于体腔淋巴瘤"]},"tr":{"name":"Primer efüzyon lenfoma","synonyms":["PEL","Vücut boşluğu temelli lenfoma"]}},"xrefs":[{"source":"ICD-10","code":"C83.8","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"2A81.9","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"697911710"},{"source":"MONDO","code":"0018842","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D054685","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1292753","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"9247","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10065857","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]},{"orpha":289644,"name":"Epstein-Barr virus-associated malignant lymphoproliferative disorder","classifications":[202]},{"orpha":102024,"name":"Human herpesvirus 8-related disorder","classifications":[202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adult"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":200.0,"area":"Worldwide","status":"Validated","source":"[EXPERT]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0003270","label":"Abdominal distention","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002585","label":"Abnormality of the peritoneum","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012191","label":"B-cell lymphoma","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002094","label":"Dyspnea","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001698","label":"Pericardial effusion","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002202","label":"Pleural effusion","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002721","label":"Immunodeficiency","frequency":"Frequent (79-30%)","criteria":null}]}}