A Rare Cause of Extreme Leukocytosis: Primary Plasma Cell Leukemia with High-risk Cytogenetics

Abstract

Abstract Background Primary plasma cell leukemia (pPCL) is a rare and highly aggressive plasma cell disorder. Extreme leukocytosis is an infrequent finding in pPCL. Case presentation: We reported an 82-year-old female patient who presented with marked leukocytosis (61.900/µl) and was diagnosed with pPCL. Plasma cells were found to have t(14;20) and 1q21 amplification. Following partial response after two cycles of bortezomib/dexamethasone combination, central nervous system (CNS) relapse occurred. Due to the advanced age and frailty, no further therapy could be administered. She had a fulminant disease course and died within one month of the CNS involvement. Conclusion PCL should be included in the differential diagnosis of leukocytosis.