{"orpha":289666,"name":"Plasmablastic lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["PBL"],"definition":"A rare aggressive B-cell non-Hodgkin lymphoma characterized by neoplastic cells resembling B immunoblasts or plasmablasts with a CD20-negative plasmacytic phenotype. The tumor may occur in the oral cavity, the gastrointestinal tract, or other, predominantly extranodal, sites and is typically associated with immunodeficiency or -suppression. The tumor cells are EBV-positive in most cases. Patients often present with disseminated bone involvement. Paraproteinemia may also be detected. Prognosis is generally poor.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-289666/","orphanet_url":"https://www.orpha.net/en/disease/detail/289666","names":{"fr":{"name":"Lymphome plasmoblastique","synonyms":["PBL"],"definition":"Lymphome non hodgkinien à cellules B agressif et rare caractérisé par des cellules néoplasiques ressemblant aux immunoblastes B ou aux plasmablastes avec un phénotype plasmocytaire CD20-négatif. La tumeur peut se développer dans la cavité buccale, le tractus gastro-intestinal ou toucher d'autres sites, principalement extranodaux, et est généralement associée à une immunodéficience ou à une immunodépression. Les cellules tumorales sont EBV-positives dans la plupart des cas. Les patients présentent souvent une atteinte osseuse disséminée. Une paraprotéinémie peut également être détectée. Le pronostic est généralement réservé."},"de":{"name":"Lymphom, plasmoblastisches","synonyms":["PBL"],"definition":"Ein seltenes aggressives B-Zell-Non-Hodgkin-Lymphom, das durch B-Zell-ähnliche neoplastische Zellen oder Plasmazellen mit CD20-negativem Plasma-Phänotyp gekennzeichnet ist. Der Tumor kann in der Mundhöhle, im Gastrointestinaltrakt oder an anderen, vorwiegend extranodalen Lokalisationen auftreten und ist typischerweise mit einer Immundefizienz oder -suppression assoziiert. Die Tumorzellen sind meist EBV-positiv. Die Patienten weisen häufig eine disseminierte Knochenbeteiligung auf. Auch eine Paraproteinämie kann beobachtet werden. Die Prognose ist in der Regel schlecht."},"es":{"name":"Linfoma plasmablástico","synonyms":["PBL"],"definition":"Es un linfoma no Hodgkin de células B agresivo poco frecuente caracterizado por células neoplásicas similares a inmunoblastos o plasmablastos B con un fenotipo plasmocítico negativo para CD20. El tumor puede aparecer en la cavidad oral, el tracto gastrointestinal, o en otros sitios, predominantemente extraganglionares, asociando típicamente inmuno -deficiencia o -supresión. Las células tumorales son positivas para el VEB en la mayoría de los casos. Los pacientes se presentan, a menudo, con afectación ósea diseminada. También se puede detectar paraproteinemia. Por lo general, el pronóstico es desfavorable."},"it":{"name":"Linfoma plasmoblastico","synonyms":["PBL"],"definition":"Si tratta di un linfoma aggressivo e raro non Hodgkin a cellule B, caratterizzato da cellule neoplastiche simili agli immunoblasti B o ai plasmablasti con un fenotipo plasmocitico CD20-negativo. Il tumore può localizzarsi nel cavo orale, nel tratto gastrointestinale o in altre sedi, per lo più extranodali, e in genere si associa ad immunodeficienza o immunosoppressione. Nella maggior parte dei casi le cellule tumorali sono EBV-positive. Spesso i pazienti presentano un diffuso coinvolgimento osseo. Può associarsi a paraproteinemia. In genere la prognosi è sfavorevole."},"nl":{"name":"Plasmablastisch lymfoom","synonyms":["PBL"],"definition":"Een zeldzaam agressief non-Hodgkinlymfoom van B-cellen, gekarakteriseerd door neoplastische cellen gelijkend op B-immunoblasten of plasmablasten met een CD20-negatief plasmacytair fenotype. De tumor kan voorkomen in mondholte, maag-darmstelsel, of andere, hoofdzakelijk extranodale plaatsen, en is doorgaans geassocieerd met immuundeficiëntie of immuunsuppressie. De tumorcellen zijn in de meeste gevallen EBV-positief. Patiënten vertonen vaak gedissemineerde betrokkenheid van bot. Paraproteïnemie wordt mogelijk ook gedetecteerd De prognose is doorgaans slecht."},"pt":{"name":"Linfoma plasmablástico","synonyms":["PBL"]},"pl":{"name":"Chłoniak plazmablastyczny","synonyms":["PBL"]},"cs":{"name":"Plazmablastický lymfom","synonyms":["PBL"]},"uk":{"name":"Плазмоцитарна лімфома","synonyms":["PBL"],"definition":"A rare aggressive B-cell non-Hodgkin lymphoma characterized by neoplastic cells resembling B immunoblasts or plasmablasts with a CD20-negative plasmacytic phenotype. The tumor may occur in the oral cavity, the gastrointestinal tract, or other, predominantly extranodal, sites and is typically associated with immunodeficiency or -suppression. The tumor cells are EBV-positive in most cases. Patients often present with disseminated bone involvement. Paraproteinemia may also be detected. Prognosis is generally poor."},"zh":{"name":"浆母细胞淋巴瘤","synonyms":["浆母细胞淋巴瘤"]},"tr":{"name":"Plazmablastik lenfoma","synonyms":["PBL"]}},"xrefs":[{"source":"ICD-10","code":"C83.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2A81.2","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"803046659"},{"source":"MONDO","code":"0017347","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D000069293","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3472614","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"12125","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10065039","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]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}