A striking response of plasmablastic lymphoma of the oral cavity to bortezomib: a case report

Abstract

BackgroundPlasmablastic lymphoma (PBL) is a rare and aggressive subtype of non-Hodgkin diffuse large B-cell lymphoma originally with a predilection to the oral cavity of patients infected with HIV. However, PBL of extraoral sites possesses clinicopathological characteristics distinct from oral PBL. Recently, therapeutic approaches using a proteasome inhibitor bortezomib to PBL of extraoral sites have been reported. We present a PBL patient with a bulky tumor of the oral cavity, who dramatically responded to bortezomib.Case PresentationThe patient was a 58 year-old Japanese male, who presented with a rapidly progressive history of a swelling on his left cheek and restricted mouth opening. He did not have a history or evidence of immunosuppression including HIV infection. A computed tomography demonstrated a bulky tumor in the oral cavity without enlarged lymph nodes. The tumor showed the proliferation of large lymphoid cells with centroblastic morphology, which were positive for CD138, CD38, CD56 and MUM-1, and negative for CD20, CD79a, BCL-6 and HHV8. The Ki-67 proliferation index was almost 100 %. Neither osteolytic lesions nor M-protein was observed. One week after the initiation of bortezomib, a marked regression of the oral tumor was obtained.ConclusionsThus, our case demonstrated the effectiveness of bortezomib on PBL of the oral cavity as well as the extraoral sites.

Recent insights in the pathogenesis of post-transplantation lymphoproliferative disorders

“…Reanalysis of our gene expression data (3 EBV + PT-PBL vs 20 EBV + PT-DLBCL, fold change 2, FDR < 0.05[ 95 ]) confirmed enhanced MYC signaling and demonstrated unfolded protein response endoplasmic reticulum stress signaling in PBL (unreported data). These findings provide an explanation for the success of bortezomib treatment in PBL case reports[ 96 , 97 ] and suggest that BET bromodomain inhibitors may represent a potential new therapeutic strategy, as has been successfully demonstrated in experimental models of multiple myeloma[ 98 ].…”

Gastrointestinal Tract Plasmablastic Lymphoma in HIV-Infected Adults: A Histopathological Review

“…Similar features were reported in a previous case on oral cavity PBL with predominant centroblastic morphology. 35 Hirosawa et al 35 reported on a case of oral cavity PBL that showed proliferation of large cells with centroblastic morphology. The observation of predominant centroblasts in a case of gastrointestinal tract PBL in the current study has not been previously described.…”

Multidetector Computed Tomography Features of Plasmablastic Lymphoma of the Oral Cavity in an HIV-Positive Patient

“…Oral PBL shows a male predilection with 54.1 average age [9]. Plasmablastic lymphoma has been reported in the elderly patients who are not immuno-compromised [11,12].…”