The biology and treatment of plasmablastic lymphoma

Abstract

Plasmablastic lymphoma (PBL) is an aggressive lymphoma commonly associated with HIV infection. However, PBL can also be seen in patients with other immunodeficiencies as well as in immunocompetent individuals. Because of its distinct clinical and pathological features, such as lack of expression of CD20, plasmablastic morphology, and clinical course characterized by early relapses and subsequent chemotherapy resistance, PBL can represent a diagnostic and therapeutic challenge for pathologists and clinicians alike. Despite the recent advances in the therapy of HIV-associated and aggressive lymphomas, patients with PBL for the most part have poor outcomes. The objectives of this review are to summarize the current knowledge on the epidemiology, biology, clinical and pathological characteristics, differential diagnosis, therapy, prognostic factors, outcomes, and potential novel therapeutic approaches in patients with PBL and also to increase the awareness toward PBL in the medical community.

EBV‐positive diffuse large B‐cell lymphoma of the elderly: 2016 update on diagnosis, risk‐stratification, and management

“…Plasmablastic lymphoma (PBL): PBL is an aggressive lymphoma with immunoblastic morphology and plasmacytic immunophenotype [17,18]. The lymphoma cells typically are of large size with round-tooval centrally or eccentrically located nucleus, dispersed chromatin, prominent single nucleolus, and amphophilic cytoplasm with perinuclear hof.…”

Orbital plasmablastic lymphoma

“…This neoplasm, a rare and aggressive subtype of DLBCL, has more frequently been described originating from the oral and sinonasal of HIV‐positive males 1, 2, but has also been reported in immunocompetent patients 2, 3, 4. In this case, the primary lesion appears to be within the orbital mucosa.…”

“…In this case, the primary lesion appears to be within the orbital mucosa. There is no standardized therapy and most published cases included systemic treatment with an individualized intensive chemotherapy regime similar to that of other lymphomas 1, 2. Prognosis is poor with an overall median survival of 8 months 5.…”

Transformation of a previously diagnosed diffuse large B‐cell lymphoma to plasmablastic lymphoma

“…However, MYC rearrangements and EBV infection have emerged as possible contributors for the development of PBL. MYC rearrangements would prevent physiological apoptotic cell death while EBV infection would favor plasmablastic transformation of mature B-cells but blocking further plasmacytic differentiation [1].…”