Nisha Patel , Eric D. Hsi
Intravascular Large B-Cell Lymphoma (IVLBCL) is a rare, extranodal, non-Hodgkin lymphoma characterized by selective growth within vessels. Recent phenotypic and genetic studies have shed insight into disease pathogenesis and potential therapeutic targets. Frequently mutated genes detected include MYD88 L265P, CD79B and PIM1. This molecular profile shows similarities to diffuse large B-cell lymphomas with MCD/Cluster 5 genetic signature, a finding also shared with primary central nervous system lymphoma and primary diffuse large B-cell lymphoma of the testis. A subset of IVLBCL cases also express programmed cell death protein 1 (PD-1) ligands, PD-L1 and/or PD-L2 and harbor underlying PD-L1/PD-L2 genetic alterations. Emerging reports suggest other immune evasion mechanisms may also be exploited, including alterations in Major Histocompatibility Complex (MHC). Although data is still limited, in part due to the rarity of IVLBCL, recent studies have expanded our understanding into the disease biology and provide rationale for potential targeted therapies.
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