Plasmablastic Lymphoma

Watchlist

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Retrieved

2021-01-23

Source

Orphanet

Trials

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Genes

MYC, IGH, MS4A1, CD38, KRT20, SDC1, PRDM1, MME, ZC3H12D, TBC1D9, ABCB1, PTPRC, RAG2, TRBV20OR9-2, VDR, HSPB3, IL17C, SMUG1, EBF3, PRDX1, CD274, FOXP3, CD320, RTEL1, PWWP3A, PAX5, ALK, NOTCH1, NCAM1, BCL2, BCL6, TNFRSF8, CDKN2A, CTNNB1, MTOR, HSPB1, HSPB2, IL2, IL4, IL6, IL10, IL13, IRF4, KIT, SMAD3, ASS1, H3P10

Drugs

Allogeneic multi-virus specific T lymphocytes targeting BK virus, cytomegalovirus, human herpesvirus-6, Epstein Barr virus and adenovirus, Iodine (131I) tositumomab, haematopoietic stem cells and blood progenitors umbilical cord-derived expanded with (1R, 4R)-N1-(2-benzyl-7-(2-methyl-2H-tetrazol-5-yl)-9H-pyrimido[4,5-b]indol-4-yl)cyclohexane-1,4-diamine dihydrobromide dihydrate

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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.