Primary Plasma Cell Leukemia: A Retrospective Study of a Rare Disease From Tertiary Cancer Centre From India.

Plasma cell leukemia (PCL) is an aggressive rare leukemic variant of multiple myeloma (MM). We aim to present 4 years data on clinical profile and treatment outcomes of Primary PCL (PPCL) patients treated at tertiary care cancer centre from Northern India. To analyse response and safety profile of a PPCL with or without stem cell transplantation. Retrospectively reviewed and analysed PPCL patient's data at our centre from January-2013 to June-2017. Total 11 PPCL patients diagnosed among 240 MM patients during study period. Eight were males. Only 10 patients were started on treatment. Four (n = 4/10) patients underwent stem cell transplantation. Overall response rate was 70% (n = 7). Eleven culture positive bacterial infections (bloodstream = 2, urinary tract = 3; pulmonary = 6) were recorded. Four patients had fungal infections. One patient had Herpes Zoster infection. Relapse rate of entire cohort was 50% (n = 5). Median PFS and OS of entire cohort was 11 months (95% confidence interval 6.3-15.6) and 21 months (95% C.I. 1-49.8) respectively. The estimated PFS and OS at 1 year of transplanted versus nontransplanted patients were 71% + 24% versus 0% (P = 0.96) and 71% + 24% versus 15% + 19% (P = 0.234) respectively. Treatment with PIs + IMAs followed by transplants (single/double) might improve depth and duration of remission and OS. Patients should be treated with indefinite maintenance therapy to control disease. © Indian Society of Hematology and Blood Transfusion 2019.