A Rare Presentation of HIV-Negative Plasmablastic Lymphoma: A Diagnostic Dilemma

Abstract

Plasmablastic lymphoma (PBL) and plasmablastic plasma cell myeloma (PCM) have many overlapping characteristics. Clinical correlation can help make the distinction between the two entities. Human immunodeficiency virus- (HIV-) negative PBL is a rare disease, making the diagnosis more challenging. While there is no standard of care for PBL, current recommendations include dose-adjusted EPOCH (etoposide, vincristine, doxorubicin, cyclophosphamide, and prednisone), with or without bortezomib. We report an aggressive case of HIV-negative plasmablastic lymphoma and discuss the challenge in establishing a diagnosis. We review the literature regarding this disease and current recommendations for treatment.

Testicular Plasmablastic Lymphoma in an HIV-Negative Patient: A Rare Case Presentation

“…In contrast, features that are associated with poor outcome include late stage of the Ann Arbor classification system, patients who test negative for EBV and cases not responsive to treatment. 9,17 Our patient presented with several poor prognostic factors such as late stage disease (stage III) and HIV-negative PBL.…”

“… 1 , 2 , 7 Furthermore, HIV-positive patients are generally diagnosed at an earlier age with PBL. 1 , 8 , 9 About a third of HIV-negative patients have some form of iatrogenic immunosuppression, for example, post organ transplantation. 7 , 8 , 10 It occurs mostly in the oral cavity, nasal cavity, skin, gastrointestinal tract, and less commonly in the testicles.…”

“… 7 , 8 , 10 It occurs mostly in the oral cavity, nasal cavity, skin, gastrointestinal tract, and less commonly in the testicles. 1 , 9 , 11 …”

“… 12 Molecular and genetic studies suggest that it arises from activated B cells in transition from immunoblast to plasma cell that fail to undergo apoptosis in the post germinal center. 1 , 4 EBV infection has also been reported to be associated with PBL 8 - 10 and it may be one of the distinguishing factors between PBL and PCM. 13 , 14 Due to several mechanisms associated with EBV antigens, EBV infection prevents apoptosis of B-cells.…”

Lymphome plasmablastique de la muqueuse nasal isolé chez un patient immunocompétent en rémission complète après traitement multimodale: a propos d'un cas Africain et revue de la littérature

“…Vu la rareté du lymphome plasmablastique, il n’y a pas de standard de traitement et la plupart des lymphomes sont résistant au traitement avec un mauvais pronostic. Plusieurs schémas thérapeutiques ont été utilisés selon les cas publiés, incluant EPOCH, CODOX-M/IVAC et hyper-CVAD, vu que le CHOP n’est pas jugé efficace [ 9 ]. Notre patient avait reçu 6 cures CHOEP suivis d’une radiothérapie après décision de RCP avec rémission complète.…”