九例人免疫缺陷病毒阴性的浆母细胞淋巴瘤临床特征及文献复习

OBJECTIVE: To evaluate the clinical features and outcomes of HIV-negtive plasmablastic lymphoma (PBL). METHODS: Nine patients with HIV-negtive PBL were diagnosed and treated between January 2006 and January 2016. The clinical and follow-up data were analyzed retrospectively. RESULTS: The median age was 56 years (range 30–77 years) with a male-to-female ratio of 2∶1. Nobody had underlying diseases associated with immunosuppression. Primary extra nodal diseases were observed in 7 cases and only 1 patient had oral involvement. Two patients were in earlystage and 7 in advanced stage by the Ann Arbor stage system. Ki-67 index was 60%–90% in the 9 cases, and 80% or higher in 7 cases. Epstein-Barr virus-encoded RNA expression (EBER) was detected in 4 cases, and 2 of them were positive. Chemotherapy was documented in 9 patients, from which 8 received the cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP)-like regimens as the first-line chemotherapy and responses were observed in 5 (1 complete, 4 partial responses). Three elderly patients were treated with CHOP combined with thalidomide, and 2 of them achieved partial responses. One patient, failed three chemotherapy regimens, accepted thalidomide combined with etopside and achieved stable disease for 10 months. One patient with early stage had disease-free survival of 61.9 months after treatment. The other eight patients experienced recurrence or progression after the first-line chemotherapy, and 6 of them died of disease progression within 2 years after the diagnosis. CONCLUSION: The HIV-negative PBL patients in this study did not have an apparent association with immunosuppression. Primary extra nodal diseases were common, but only 1 patient had oral involvement. Most patients had advanced stage and poor prognosis. Effectiveness of thalidomide in the treatment of PBL deserves further investigation.