Extraosseous Plasmacytoma Confers Poor Outcomes in Primary Plasma Cell Leukemia.

BACKGROUND

Primary plasma cell leukemia (PPCL) is one of the most aggressive diseases in oncohematology. Due to its low incidence, there is no established standard treatment, and it is considered an incurable disease.

AIMS: This study presents our experience and outcomes of 92 cases of PPCL between January 2011 and December 2020.

METHODS

Fat pad biopsy was positive for Congo red in six (35%) of 17 patients, solitary extraosseous plasmacytoma was observed in 30 (32.5%), and bone marrow fibrosis was identified in 81 (88%) patients.

RESULTS

While most patients (69.5%) received conventional chemotherapy combinations, 30.5% underwent proteasome inhibitor-based treatment and/or autologous stem cell transplantation (auto-SCT). In this series, we report that the presence of extraosseous plasmacytoma, treatment failure, and therapy with classic chemotherapy agents were independent covariates associated with poor survival.

CONCLUSIONS

In PPCL, extraosseous plasmacytoma, treatment failure, and treatment with conventional chemotherapy were associated with poor outcomes.