Rare presentation of orbital plasmablastic lymphoma with oral cavity involvement in an HIV-negative patient

Abstract

Plasmablastic lymphoma is described as a subtype of non-Hodgkin's lymphoma under the category of diffuse large B-cell lymphoma. It is classified by WHO as HIV-associated lymphoma of the oral cavity. Several cases have been reported in non-HIV patients with extra-oral involvement. The characteristic immunohistochemical markers are generally positive for CD138, CD38 and MUM1/IRF4, and negative or weakly positive for pan B-cell markersCD20, CD79a, PAX-5 and BCL-6. We report a rare case of orbital plasmablastic lymphoma with oral and oropharyngeal involvement where the immunohistochemical markers were positive for CD138, CD43, CD45, CD79a and MUM1. A small subset of markers was weakly positive for CD20, CD30 and κ, and negative for CD10, BCL-6, CD4, CD56 and Epstein-Barr virus-encoded small RNA (EBER). Our case reinforces the fact that plasmablastic lymphoma is a different entity of non-Hodgkin's lymphoma that cannot simply be classified under diffuse large B-cell lymphoma. It demands modification of the WHO classification.

Case Report: Conjunctival Chemosis as a Presenting Sign of Plasmablastic Lymphoma in a HIV Negative Patient

“…We performed a thorough literature search of all reported cases of orbital plasmablastic lymphoma and found only 13 cases. Of those, only four occurred in HIV-negative individuals 5,6,12,13 . Our patient is the fifth reported case of orbital plasmablastic lymphoma in an HIV-negative patient.…”

“…Of those, only four occurred in HIV-negative individuals. 5,6,12,13 Our patient is the fifth reported case of orbital plasmablastic lymphoma in an HIV-negative patient.…”

“…The most common presenting clinical signs and symptoms of orbital plasmablastic lymphoma in reported cases include eye pain, reduced vision, eyelid swelling, ophthalmoplegia, conjunctival chemosis, and globe proptosis (Table 1). Conjunctival chemosis and globe proptosis are the two most common clinical signs, with conjunctival chemosis present in 8 of the 13 total cases reported in the literature and proptosis reported in all of the cases 5–13 . We report a fifth case of an HIV-negative patient with ocular adnexal involvement of unilateral conjunctival chemosis and mild globe proptosis as initial presenting signs of plasmablastic lymphoma.…”

“…[4][5][6][7][8] The most common presenting clinical signs and symptoms of orbital plasmablastic lymphoma in reported cases include eye pain, reduced vision, eyelid swelling, ophthalmoplegia, conjunctival chemosis, and globe proptosis ( all of the cases. [5][6][7][8][9][10][11][12][13] We report a fifth case of an HIV-negative patient with ocular adnexal involvement of unilateral conjunctival chemosis and mild globe proptosis as initial presenting signs of plasmablastic lymphoma.…”