Plasmablastic Lymphoma of the Oral Cavity Type as the Presenting Manifestation of HIV Infection

Plasmablastic Lymphoma of the Oral Cavity Type as the Presenting Manifestation of HIV Infection

Antonino Carbone, MD

Department of Pathology and Laboratory Medicine, Fondazione IRCCS, National Cancer Institute of Milan, Milan, Italy

The case study by Khan and colleagues1 describes an interesting case of HIV-associated plasmablastic lymphoma (PBL) of the pelvis, which occurred as the initial presentation of AIDS. A 40-year-old woman, with no medical history, presented with a large mass in the pelvis that extended from the vaginal introitus to the sacral promontory in absence of a computed tomography scan, and sigmoidoscopic evidence of enterovaginal fistula. Significant nuclear pleomorphism, with plasmacytoid morphology in some cells. PBL with plasmacytoid differentiation and diffuse large B-cell lymphoma with secretory differentiation (immunoblasts and plasmacytoid cells) could be distinguished by the presence of centroblasts in the latter. CD138 and MUM1, markers of post–germinal center/terminal B-cell/plasmacytoid differentiation, are useful in identifying the lymphoid and B-cell origin of these tumors, which show variable or negative expression of CD20 and CD45. Because of the common absence of these markers and its histologic features, PBL, in particular, can be misinterpreted as a nonlymphoid tumor.

Uncommonly, primary effusion lymphoma (PEL) may present as a solid form that predominantly involves the distal digestive tract and poses major diagnostic problems, especially when it is unassociated with body cavity effusions. The solid forms of both PEL and PBL display plasmablastic features. Demonstration of KSHV/HHV-8 presence excludes a PBL and establishes the diagnosis of a solid form of PEL. The need to investigate KSHV/HHV-8 in any plasmablastic-looking lymphoma, especially in HIV-infected patients, is relevant.9-11 As noted earlier, the prognosis of PBL had initially been very poor. However, increased survival times have been observed in HIV-infected patients with the combination of HAART and chemotherapy. Lymphomaspecific chemotherapy has included cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP); CHOP with intrathecal methotrexate, doxorubicin, cyclophosphamide, vindesine, bleomycin, and prednisone (ACVBP); and EPOCH.8 The role for rituximab, an anti-CD20 monoclonal antibody, in PBL has not been defined. PBL is a subtype of diffuse large B-cell lymphoma, and rituximab may provide some benefit, although it may not play a role in PBL therapy because CD20 is not usually expressed by PBL tumor cells. On the contrary, it is imperative to include prophylaxis against opportunistic infections for HIV patients receiving chemotherapy. A characteristic feature of PBL is its rapidly progressive clinical course. However, recent reports have noted improved survival when treatment with both HAART and appropriate chemotherapy is used, similar to outcomes of HIV-infected patients with other non-Hodgkin lymphomas.

In conclusion, the consistent association of PBL with HIV (80–90%) and immunosuppression (90%), and the apparent improved prognosis when HAART is combined with chemotherapy indicate that patients diagnosed with PBL should be tested for HIV. In fact, the diagnosis of PBL may be the presenting manifestation of HIV infection,8,12 such as in the case reported by Khan and colleagues.1

References

1. Khan M, Jakate S, Komanduri S. Rare AIDS-associated plasmablastic lymphoma as the initial presentation of AIDS. Clin Adv Hematol Oncol. 2010;1:55-57.

2. Carbone A, Cesarman E, Spina M, Gloghini A, Schulz TF. HIV-associated lymphomas and gamma-herpesviruses. Blood. 2009;113:1213-1224.

3. Navarro WH, Kaplan LD. AIDS-related lymphoproliferative disease. Blood. 2006;107:13-20.

4. Delecluse HJ, Anagnostopoulos I, Dallenbach F, Hummel M, Marafioti T, Schneider U, et al. Plasmablastic lymphomas of the oral cavity: a new entity associated with the human immunodeficiency virus infection. Blood. 1997;89:1413-1420.

5. Teruya-Feldstein J, Chiao E, Filippa DA, Lin O, Comenzo R, Coleman M, et al. CD20-negative large-cell lymphoma with plasmablastic features: a clinically heterogenous spectrum in both HIV-positive and -negative patients. Ann Oncol. 2004;15:1673-1679.

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8. Riedel DJ, Gonzalez-Cuyar F, Zhao XF, Redfield RR, Gilliam BL. Plasmablastic lymphoma of the oral cavity: a rapidly progressive lymphoma associated with HIV infection. Lancet Infect Dis. 2008;8:261-267.

9. Teruya-Feldstein J. Diffuse large B-cell lymphomas with plasmablastic differentiation. Curr Oncol Rep. 2005;7:357-363.

10. Carbone A, Gloghini A, Vaccher E, et al. Kaposi’s sarcoma–associated herpesvirus/human herpesvirus type 8-positive solid lymphomas: a tissue-based variant of primary effusion lymphoma. J Mol Diagn. 2005;7:17-27.

11. Carbone A, Gloghini A, Vaccher E, Marchetti G, Gaidano G, Tirelli U. KSHV/HHV-8 associated lymph node based lymphomas in HIV seronegative subjects. Report of two cases with anaplastic large cell morphology and plasmablastic immunophenotype. J Clin Pathol. 2005;58:1039-1045.

12. Sarode SC, Zarkar GA, Desai RS, Sabane VS, Kulkarni MA. Plasmablastic lymphoma of the oral cavity in an HIV-positive patient: a case report and review of literature. Int J Oral Maxillofac Surg. 2009;38:993-999.