HIV‐associated plasmablastic lymphoma: Lessons learned from 112 published cases

Abstract

Plasmablastic lymphoma (PBL) is a distinct subtype of non-Hodgkin B-cell lymphoma, originally described with a strong predilection to the oral cavity of human immunodeficiency virus (HIV)-infected individuals. Data regarding patient age and gender, HIV status, initiation of and response to highly active antiretroviral therapy (HAART), tumor extent, pathology, treatment, and outcome were extracted from 112 cases of PBL identified in the literature. The median age at presentation was 38 years with a male predominance of 7:1, and the median CD41 count was 178 cells/mm 3 . PBL presented on average 5 years after diagnosis of HIV. Common primary sites of presentation included the oral cavity, gastrointestinal tract, and lymph nodes. Most cases presented with either stage I or stage IV disease. There was a variable expression of B-cell markers in tumor cells, but plasma cell markers were expressed in all cases. EBV was detected in 74%. Chemotherapy was used to treat 55% patients and was combined with radiotherapy in 21% cases. Complete response was obtained in 66% of treated cases; the majority of these responses were seen after CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone). The refractory/relapsed disease rate was 54%. Death occurred in 53% of patients, with a median overall survival of 15 months. Sex, CD41 count, viral load, clinical stage, EBV status, primary site of involvement, and use of CHOP failed to show an association with survival. PBL is an aggressive B-cell lymphoma that presents in both oral and extra-oral sites of chronically HIV-infected immunosuppressed young men. Am. J. Hematol. 83:804-809, 2008. V

Large Cell Lymphoma: Correlation of HIV Status and Prognosis with Differentiation Profiles Assessed by Immunophenotyping

“…The mean CD4 count in patients with PBL in our study is in-keeping with that quoted by Castillo et al 153 (<200/mm 3 ) and lower than the CD4 count of 300/mm 3 reported in a case study by Flaitz et al 153 154 Two cases of PBL occurred in the oral cavity and seven were diagnosed at extra-oral and extranodal topographic sites. While classic blast-like morphology was evident in the tumours occurring in the oral cavity, morphologic features of plasmacytic Overall, EBV LMP1 immunoexpression was detected in 3/9 PBL cases.…”

Plasmablastic Lymphoma of Small Intestine: A Rare Case Report with Review of Literature

“…However, in recent years, several cases of PBL have been reported at extraoral sites, including the skin, [2] subcutaneous tissue, [3] stomach, [4] anal mucosa or perianal area [5], lung [6] lymph node [7], and other regions. Small intestine has rarely been reported as a site for PBL with only five cases [8][9][10][11][12] reported in the literature before, present case being the sixth case.…”

Plasmablastic lymphoma: One or more entities?

“…In this issue of the American Journal of Hematology, Castillo et al [3] present the first extensive literature review of the epidemiology, clinical findings, pathology, treatment, and outcome of 112 patients with HIV-associated PBL. The authors point out that HIV-associated PBL tends to affect young men with CD41 counts less than 200 cells/mm 3 .…”

“…The authors point out that HIV-associated PBL tends to affect young men with CD41 counts less than 200 cells/mm 3 . The consistent presence of plasma cell markers and frequent absence of B-cell markers are key pathologic features of this entity [3].…”

“…Most patients are HIV infected and tumor cells are EBV positive but HHV8 negative. Most cases present in the oral mucosa, whereas a significant number of cases present in other extranodal or nodal sites [3]; (2) HIVassociated lymphoma showing IB morphology with plasmacytoid differentiation. These lymphomas can be either systemic or primary central nervous system lymphomas; (3) tumors defined as PBL with plasmacytic differentiation [12].…”