Cutaneous Plasmablastic Lymphoma in an HIV-Positive Male: An Unrecognized Cutaneous Manifestation

Abstract

Plasmablastic lymphoma (PBL) is a rare and relatively new entity originally described in HIV-infected individuals. This subset of Epstein-Barr-virus (EBV)-related non-Hodgkin lymphomas is now regarded as a distinct clinicopathological category of AIDS-associated lymphomas occurring preferentially in the oral cavity and showing a poor prognosis. We describe for the first time an EBV-associated PBL with an isolated cutaneous distribution on the lower extremities in an HIV-infected heterosexual male and point to the unique clinical, morphological and immunophenotypic characteristics of this lymphoma. The patient presented with fast growing solid and livid nodules on both legs. The large, blastic tumor cells showed the following immunophenotype: CD138+, CD45+, CD20–, CD10–, CD3–, CD30–, bcl-2–, bcl-6–, LMP-1– and EMA–. The proliferation fraction (Mib-1) was >90%. EBV association was demonstrated by in situ hybridization (EBV-encoded RNAs 1/2). Polymerase-chain-reaction-based DNA analysis demonstrated a clonal IgH rearrangement in the absence of a bcl-2/IgH translocation. PBL in HIV patients may occur not only in the oral cavity, but can probably involve any other organs including the skin.

WHO-EORTC classification for cutaneous lymphomas

“…Plasmablastic lymphomas are seen almost exclusively in the setting of HIV infection or other immune deficiencies. [170][171][172] Some of these cases had only skin lesions at presentation. Rare cases of primary cutaneous T-cell/histiocyte-rich B-cell lymphoma, characterized by the presence of large scattered B cells in a background of numerous reactive T cells, have been reported.…”

Extra-oral plasmablastic lymphoma: report of a case and review of literature

“…Although the World Health Organization (WHO) classification listed PBL as a variant of DLBCL and HIV-related lymphoma, and named it plasmablastic lymphoma of the oral cavity because of the common occurrence in the oral cavity, more and more extra-oral PBLs have been reported [6][7][8][9][10][11][12][13][14], suggesting that this classification may need to be revised.…”

Plasmablastic lymphoma of the oral cavity, a B cell-derived lymphoma associated with HIV infection: a case series

“…Extra-oral sites include maxillary sinus [10], pharynx [22], stomach [12,23], bowel [12,13,24], lung [12,25], spermatic cord [13], perianal area [12-14, 22, 26, 27], bone or bone marrow [12,14], skin [12,14,[28][29][30][31], soft tissues [32], and lymph nodes [13,14,27,33]. More than 20% of the extra-oral PBL locations involve lymph nodes [22].…”

“…Mitotic figures and apoptotic bodies are frequent and many tingible macrophages are traced, resulting to a ''starry-sky'' appearance [1,10,21,26,28]. There is also a tendency of PBL to infiltrate the bone marrow [2,36].…”