Background:Human immunodeficiency virus-negative plasmablastic lymphoma is an extremely rare entity. Its clinicopathological features, optimal treatment strategy and prognostic factors are still obsure.
Aim:To investigate the clinicopathological characteristics and prognostic factors of HIV negative PBL by retrospective analysis.
Methods:An extensive search was performed in English literature within Pubmed database using the keywords: plasmablastic lymphoma and human immunodeficiency virus negative or immunocompetent. 114 patients’ data from 52 articles between 1997 and 2014 were analyzed.
Results:
The mean age at diagnosis was 58.90 years (range, 2-86). HIV negative PBL showed a predilection of elderly individuals (patients elder than 60 years: 56.14% ) and affected more male than female (M：F=2.29:1). Ann arbor stage IV patients accounted for 39.22 % while bone marrow involvement was less frequent (12.79%). Ki-67 index was high with a mean expression of 83%. EBV infection was common, positive in 58.70% patients while HHV-8 infection was rare, positive in only 7.55% patients. 28.16% of patients had immunosuppression. The median overall survival was 8 months. The 1-year, 2-year, 3-year, 4-year and 5-year survival rates were 42.5%, 18.75%, 11.25%, 5% and 2.5% respectively. Age, gender, primary sites, EBV infection and Ann arbor stage showed no strong relation with OS while immunosuppression could predict a poorer OS. Either CR or PR was superior to refractory group in OS (p< 0.0001 and p=0.004 respectively). For stage Ⅰ patients, the application of radiotherapy didn’t improve the OS.
Conclusions:
HIV negative PBL is a distinct entity likely occurring in elderly and immunosuppressed individuals. Immunosuppression status and refractory to treatment are poor prognostic factors of OS.