Plasma cell dyscrasias represent 1.4-2 % of all malignancies and among hematologic malignancies; it constitutes 10 % of the tumors. Plasma cell dyscrasias are composed of multiple myeloma, primary and secondary plasma cell leukemia. Primary plasma cell leukemia (pPCL) is a rare and aggressive disease, represents 1-4 % of plasma cell dyscrasias. The prognosis of this is very poor with median survival of 8- 11 months in different reported series. We are reporting a study from our hospital over a period of ten years, in which pPCL was found in 1.8% of multiple myeloma patients, with slight male predominance and earlier age than multiple myeloma, and all had high disease burden with high LDH, β2 microglobulin, and plasmacytosis. This disease had very aggressive behavior especially with light chain lambda disease and all patients succumbed within 8 months of treatment.
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