High-dose therapy and autologous hematopoietic cell transplantation as consolidation treatment for primary effusion lymphoma

Abstract Background Primary effusion lymphoma (PEL) is a rare subtype of non-Hodgkin lymphoma. Limited disease-free survival after chemotherapy results in poor prognoses. Outcome data of high-dose therapy followed by autologous hematopoietic cell transplantation (auto-HCT) in PEL is limited due to rarity of the disease. Patients and methods The study included 9 PEL patients from 2 major academic centers; 4 patients received auto-HCT after high-dose therapy. Eight (89%) had immunodeficiency (HIV seropositive = 7; solid organ transplant recipient = 1) at the time of diagnosis. Human herpes virus-8 by immunohistochemistry was positive in 8 samples. Anthracycline-based combination chemotherapy was used as first-line treatment for 7 patients; 4 underwent auto-HCT while in first complete remission (CR1). Results Median follow-up of surviving patients was 25 months (95% CI = 8%-29%). The 2-year progression-free (PFS) and overall (OS) survival for 8 patients who received treatment were 58% (95% CI = 22%-95%) and 73% (95% CI = 41%-100%), respectively. Moreover, the 2-year PFS and OS for patients who received auto-HCT were 50% (95% CI = 1%-99%) and 75% (95% CI = 33%-100%), respectively. Among the 4 auto-HCT recipients, all were in CR1 and the cumulative incidence of relapse was 50% (95% CI = 19%-100%). There were no deaths attributable to auto-HCT at 2 years. Conclusion Despite the small sample size, our data show that consolidative auto-HCT is safe and could be considered for eligible PEL patients after induction chemotherapy. However, a high relapse rate is concerning and warrants incorporation of new strategies to mitigate post-transplantation relapse.