Abstract
Plasmablastic lymphoma (PBL) is an aggressive subtype of non-Hodgkin's lymphoma (NHL), which frequently arises in the oral cavity of human immunodeficiency virus (HIV) infected patients. PBL shows diffuse proliferation of large neoplastic cells resembling B-immunoblasts/plasmablasts, or with plasmacytic features and an immunophenotype of plasma cells. PBL remains a diagnostic challenge due to its peculiar morphology and an immunohistochemical profile similar to plasma cell myeloma (PCM). PBL is also a therapeutic challenge with a clinical course characterized by a high rate of relapse and death. There is no standard chemotherapy protocol for treatment of PBL. Cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) or CHOP-like regimens have been the backbone while more intensive regimens such as cyclophosphamide, vincristine, doxorubicin, high-dose methotrexate/ifosfamide, etoposide, high-dose cytarabine (CODOX-M/IVAC), or dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin (DA-EPOCH) are possible options. Recently, a few studies have reported the potential value of the proteasome inhibitor bortezomib and thalidomide in PBL patients. The introduction of genes encoding artificial receptors called chimeric antigen receptors (CARs) and CAR-modified T cells targeted to the B cell-specific CD19 antigen have demonstrated promising results in multiple early clinical trials. The aim of this paper is to review the recent advances in epidemiology; pathophysiology; clinical, pathologic, and molecular characteristics; therapy; and outcome in patients with PBL.
Highlights
Plasmablastic lymphoma (PBL) is a rare lymphoma associated with immunosuppression
The presence of a spectrum of plasmacellular differentiation has been introduced in the following years and is a frequent feature of plasmablastic lymphomas outside the oral cavity [63]
PBL has a predilection for immunocompromised individuals based on its prevalence in both human immunodeficiency virus (HIV)-positive patients and in those undergoing solid organ transplantation
Summary
Plasmablastic lymphoma (PBL) is a rare lymphoma associated with immunosuppression It is strongly associated with human immunodeficiency virus (HIV) infection and often occurs within the oral cavity. PBL is seen in elderly patients with age-associated immunosuppression and other patients receiving immunosuppressive therapy; despite its predisposition for the immunocompromised patients, PBL has been diagnosed in immunocompetent patients [1, 2]. This lymphoma is considered a separate diagnostic entity, distinct from diffuse large B-cell lymphoma (DLBCL), not otherwise specified, in the 2008 World Health Organization (WHO) classification of lymphoid neoplasms [3]. Cases of PBL occurring in immunocompetent patients have been documented and increasingly described in HIVnegative patients [5,6,7]
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