Plasmablastic Lymphoma Remains Challenging to Treat, Resulting in Poor Prognosis 

In a study published in Blood, researchers sought to better characterize the disease features, patient characteristics, and the effects of immunodeficiency on survival among patients with plasmablastic lymphoma (PBL). 

PBL is a rare, aggressive form of non-Hodgkin lymphoma and is frequently associated with HIV infection. It is driven by immunodeficiency and optimal therapies have yet to receive meaningful consensus, leading to poor long-term outcomes. 

Because PBL is such a rare disorder, treatment protocols are conspicuously lacking. Notably, the 3- to 5-year overall survival remains low at around 4%. Much of the published studies regarding PBL are case reports and studies that are retrospective in nature. 

The authors of the study hence sought to expand the body of knowledge regarding PBL by conducting a retrospective analysis to assess clinical features, treatment, and outcomes in patients with PBL. The overarching goal of this study was to identify prognostic factors that may help patients achieve more promising long-term outcomes. 

This international, multicenter, retrospective study was conducted in 22 contributing sites across Canada, the United Kingdom, Singapore, and Australia. The research team included patients who had PBL as confirmed via tissue biopsy between January 1999 and December 2020. They were also interested in cases of immunosuppression-related PBL and their causes. Bone marrow involvement was assessed thoroughly. 

The study retrospectively recruited 281 patients from these 22 sites. Overall, half of the individuals (51%) had stage IV disease. A hundred and 34 individuals were staged by positron emission tomography (PET) and 92 via computerized tomography. A total of 51 of the 200 patients who underwent bone marrow biopsy were discovered to have bone marrow involvement. Of the individuals who were HIV-positive, 63 were started on antiretroviral therapy, and 25 were started on antiretroviral therapy after PBL diagnosis. 

Researchers reported that the median follow-up period was 1.15 years, during which 167 deaths occurred and 33 were lost to follow-up. The median overall survival was a mere 1.72 years. The most common cause of death was progressive PBL (n=117), followed by infection (n=19) and subsequent primary malignancy (n=10). 

Researchers also discovered that 234 patients received therapy with curative intent; 159 received standard-intensity regimes (mainly CHOP), while 75 were administered high-intensity regimens (dose-adjusted etoposide, prednisone, cyclophosphamide, and doxorubicin). The research team found that patients treated with a high-intensity regimen had a better chance of having a favorable Eastern Cooperative Oncology Group (ECOG) performance status. 

A total of 28% of patients who initially achieved a complete response after first-line treatment underwent relapse. Among the 169 patients who achieved an initial response to curative therapy, 59 relapsed or progressed. Overall, the median time to first relapse/progression from diagnosis was merely 8.4 months.

“PBL remains a relatively rare and challenging entity, with poor long-term outcomes and no standardized approaches to treatment,” the authors of the report concluded. “Future research is warranted and may include prospective studies of different regimens, incorporation of novel therapies, such as immunotherapies and targeted agents, and exploration of tailored approaches based on specific clinical, virological, or molecular features.”

This article originally appeared on Hematology Advisor