Abstract

BACKGROUND: Plasmablastic lymphoma (PBL) is a rare and aggressive form of non-Hodgkin's lymphoma with strong HIV association. Management is challenging due to lack of data regarding clinical characteristics, prognostic factors and therapeutic options. Even less is known about central nervous system (CNS) involvement and the role of intrathecal prophylaxis (IT-PX). METHODS: Retrospective review of patients with CNS involvement by PBL treated at Montefiore Medical Center 2002-2013. RESULTS: We identified 16 patients with PBL; 10 (62.5%) were HIV positive. Three (19%) had CNS involvement. Median age was 38 (24-41). At presentation, one had stage IB; two had stage IV disease. CD4 counts were 15, 164 and 434 cells/mm3. Only one of them was on antiretroviral therapy. All three received EPOCH without IT-PX. One patient was lost to follow-up after 3 cycles, but re-presented 33 months later with bulky CNS disease and systemic recurrence. He died 1 month later after treatment with WBRT. The other two developed CNS involvement within 7 months of diagnosis. One received 4 cycles of high-dose methotrexate (HD-MTX) with vincristine and intra-ommaya (IO) cytarabine (Ara-C). However, CSF sampling showed persistent disease and BEAM with autologous stem cell transplant (ASCT) was used as salvage. A second patient had just initiated second line R-ICE when leptomeningeal metastasis occurred. CNS-directed therapy included IO rituximab, MTX, Ara-C and HD-MTX. Her CSF cleared and she received BEAM/ASCT. CNS-directed treatment provided symptom-relief in both cases, but median survival was only 5 months (2-10). Of 11 treated PBL patients, only two (18%) received IT-PX. The remaining five refused chemotherapy or were lost to follow-up. CONCLUSIONS: CNS involvement is not uncommon and may be seen more often in HIV-associated PBLs. CSF-directed treatment can provide symptomatic relief and may produce cytological and radiographic responses. Its utility in preventing CNS seeding and improving survival is less clear.

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