Causes of death in primary plasma cell leukemia differ from multiple myeloma: A STROBE-compliant descriptive study based on SEER database

The primary plasma cell leukemia (pPCL) is a rare but aggressive variant of multiple myeloma (MM). Few studies have focused on the differences in the causes of death between pPCL and MM. This study aimed to compare and evaluate the causes of death of patients with pPCL and MM. The data were collected from the Surveillance Epidemiology, and End Results (SEER) database. The demographic characteristics, survival, and causes of death in pPCL and MM patients were evaluated and compared. The competing risk regression model was performed to predict the cause of death. Between 1975 and 2009, the overall mortality rate was 96.13% and 88.71% for pPCL and MM, and the median survival was 9 and 26 months, respectively. In pPCL, leukemia caused 45.05% of the deaths, followed by myeloma (38.83%). In MM, myeloma was the leading cause of death, accounting for 74.89% of the deaths. Older age at diagnosis was a risk factor for dying of leukemia in pPCL patients (HR = 1.49, 95% CI: 1.16-1.91), while older age at death was associated with reduced risk (HR = 0.67, 95% CI: 0.52-0.86). Although the survival of pPCL patients increased with time periods of diagnosis since 1975 to 2009, the risk of dying of leukemia increased with the periods. For MM, most of the demographic characteristics were found to have independently predicting influence on the cause of death. Patients with pPCL and MM had distinct causes of death. Leukemia was the leading and the most serious cause of death in pPCL patients. The demographic factors could not predict the causes of death in pPCL. More large-scale and multi-center studies are needed to evaluate the effect of novel agents in pPCL patients, especially for patients who have progressed to leukemia.