HIV-negative plasmablastic lymphoma: report of 8 cases and a comprehensive review of 394 published cases

Abstract

Background: Human immunodeficiency virus (HIV)-negative plasmablastic lymphoma (PBL) is a rare entity of diffuse large B-cell lymphoma (DLBCL). The clinicopathological features of and optimal treatment for HIV-negative PBL remain largely unknown.

Methods: To gain insight into this distinct lymphoma, we summarized the clinicopathologic characteristics of 8 unpublished HIV-negative PBLs and performed a comprehensive review of 394 published cases.

Results: Of the 8 unpublished PBLs, the median patient age was 53.0 years. Four patients presented with stage IV disease. All 8 patients showed a plasma cell-like immunophenotype. Of the six patients who received anthracycline-based chemotherapy, including two who received bortezomib, three patients achieved a continuous complete response, two patients died due to disease progression, and one patient was lost to follow-up. The other two patients achieved continuous complete response after receiving chemotherapy combined with radiotherapy and surgery. Of the 402 patients, the majority were male, with a mean age of 58.0 years. EBV infection was detected in 55.7% of the patients. The median survival times of the patients who received CHOP or CHOP-like regimens and intensive regimens were not reached and 23.0 months, respectively, and the intensive regimen did not improve the survival outcome (P=0.981). Multivariate analysis showed that EBER remained the only independent factor affecting overall survival (OS).

Conclusion: HIV-negative PBL is a distinct entity with a predilection for elderly and immunosuppressed individuals. Intensive chemotherapy had no apparent survival benefits over the CHOP regimen in terms of OS; the prognosis of this disease is poor with current chemotherapy methods, and treatment remains a challenge.

Keywords: Clinicopathological features; HIV negative; Plasmablastic lymphoma; Prognosis; Treatment.

Fig. 1. (A) The 1-year and 2-year overall survival rates were 55.9% and 44.8%, respectively (N=253). (B) The median survival time of patients receiving intensive regimens was 23.0 months, while the median survival time of those who received CHOP or CHOP-like regimen has not yet been reached. Intensive regimen did not improve the survival outcome (P=0.981). (C) Significant impact of EBV on overall survival (OS). Patients with EBV positivity showed a significantly better OS (1-year OS, 65.0% vs. 51.5%; P=0.004). (D) Significant impact of MYC rearrangement on OS. MYC rearrangement did not affect the survival outcome (1-year OS, 64.3% vs. 52.4%; P=0.729).