Viral, immunologic, and clinical features of primary effusion lymphoma

Primary effusion lymphoma (PEL) is an aggressive HIV-associated lymphoma with a relatively poor prognosis in the era of effective HIV therapy. Kaposi sarcoma herpesvirus (KSHV) is the etiologic agent, and approximately 80% of tumors are Epstein Barr virus (EBV) co-infected. A better understanding of how KSHV-related immune dysregulation contributes to the natural history of PEL will improve outcomes. We identified 20 PEL patients diagnosed 2000-2013, including 19 treated with modified infusional etoposide, vincristine, and doxorubicin with cyclophosphamide and prednisone (EPOCH) and compared their clinical, virologic, and immunologic features with 20 patients with HIV-associated diffuse large B-cell lymphoma (HIV-DLBCL) and 19 patients with symptomatic interleukin (IL)-6 related KSHV-associated multicentric Castleman disease (KSHV-MCD). We then performed survival analyses of treated PEL patients to identify prognostic factors and cancer-specific mortality. Compared to HIV-DLBCL, PEL was associated with significant hypoalbuminemia (p