Plasmablastic Lymphoma

Abstract

Plasmablastic lymphoma (PBL) is an infrequent and very aggressive form of B-cell malignancy distinguished by large neoplastic cells that mostly resemble plasmablasts and are phenotypically CD20-negative. While its initial association was with HIV, subsequent research has revealed its presence in individuals who have undergone solid organ transplants as well as in immunocompetent people. The molecular mechanisms underlying PBL are not well understood, and its diagnosis poses challenges because of its infrequent occurrence. But by accurately identifying its clinical traits, location, and morphological aspects, a proper diagnosis of PBL within the range of CD20-negative large B-cell lymphomas can be made. When tumor cells don't express CD20 or PAX5 and have the plasmacytic differentiation markers CD38, CD138, MUM1/IRF4, Blimp1, and XBP1, a PBL diagnosis can be made. PBL should be distinguished from other CD20-negative B-cell neoplasms such as primary effusion lymphoma, anaplastic lymphoma kinase-positive (ALK) large B-cell lymphoma, and LBCL. It is usually associated with infections caused by the Epstein-Barr virus as well as rearrangements in the MYC gene. In spite of the fact that we have improved our knowledge of this disease, the prognosis is still unfavorable, and the overall survival rate is low. There is no defined standard of care appropriate for this lymphoma. There have been a number of different chemotherapy combinations used, with relatively little difference in the results. As an obvious consequence of this, new strategies based on employing novel molecules start to appear in clinical practice in an effort to improve the patient's prognosis.