Primary plasma cell leukemia

BACKGROUND: Primary plasma cell leukemia (PCL) is a rare plasma cell disorder, and current knowledge regarding survival in this disease is limited to small series of patients. Although there has been significant improvement in the survival of patients with multiple myeloma (MM) over the past few decades, it is not known whether there has been a similar trend for PCL. METHODS: The authors analyzed the Surveillance, Epidemiology, and End Results (SEER) database to evaluate the characteristics and survival of patients who had PCL compared with those of patients who had MM. RESULTS: Among 291 patients with PCL, the median age was 67 years (range, 19-98 years), the distribution of men and women was nearly equal, and the majority of patients were white (79.4%). The median overall survival (OS) was 4 months and the median disease-specific survival (DSS) was 6 months for patients with PCL; the 1-year, 2-year, and 5-year OS rates were 27.8%, 14.1%, and 6.4%, respectively. There were no survival differences noted according to sex or race. Patients agedor=60 years (median OS, 7 months vs 3 months; P=.007), although the 5-year OS was equally poor in both groups (6.3% vs 6.4%, respectively). During the same period, 49,106 patients with MM were identified. Unlike MM, in which there has been a modest but statistically significant improvement in OS and DSS noted over time, no significant improvement was evident for PCL. CONCLUSIONS: The poor long-term outcome and the lack of survival improvement in PCL suggest the need for better therapeutic options for these patients.