[Clinical features and outcomes: analysis of 9 cases of HIV-negtive plasmablastic lymphoma]
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1Department of Medical Oncology, Beijing Key Laboratory of Clinical Study on Anticancer Molecular Targeted Drugs, National Cancer Center/Cancer Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100021, China.
Insights
HIV-negative plasmablastic lymphoma (PBL) often presents as advanced-stage disease without immunosuppression, showing a poor prognosis. Thalidomide may offer treatment benefits, warranting further investigation for this rare lymphoma subtype.
Area of Science:
- Oncology
- Hematology
- Pathology
Background:
- Plasmablastic lymphoma (PBL) is an aggressive non-Hodgkin lymphoma.
- While often associated with HIV, a subset of patients are HIV-negative.
- Clinical features and outcomes of HIV-negative PBL require further elucidation.
Purpose of the Study:
- To evaluate the clinical characteristics and treatment outcomes of HIV-negative plasmablastic lymphoma.
- To identify prognostic factors and potential therapeutic strategies for this patient group.
Main Methods:
- Retrospective analysis of clinical and follow-up data from nine HIV-negative PBL patients diagnosed between 2006 and 2016.
- Review of patient demographics, disease presentation, staging (Ann Arbor system), Ki-67 index, and Epstein-Barr virus-encoded RNA (EBER) expression.
- Analysis of treatment regimens, including cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP)-like chemotherapy and thalidomide-based therapies, and patient responses.
Main Results:
- The median age was 56 years, with a 2:1 male-to-female ratio; no patients had immunosuppressive conditions.
- Primary extranodal disease was common (7/9 cases), but oral involvement was rare (1/9).
- Most patients presented with advanced-stage disease (7/9) and high Ki-67 indices (60-90%).
- First-line CHOP-like chemotherapy yielded responses in 5/8 patients, but 8/9 experienced recurrence or progression, with 6 deaths within 2 years.
- Thalidomide-based regimens showed partial responses in 2/3 elderly patients and stable disease in one refractory patient.
Conclusions:
- HIV-negative PBL typically presents as advanced-stage disease without immunosuppression and carries a poor prognosis.
- Extranodal involvement is frequent, contrasting with typical oral localization in HIV-associated PBL.
- The potential efficacy of thalidomide in treating HIV-negative PBL warrants further clinical investigation.
Abstract:
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.
