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  • Title: Plasma cell leukemia in North India: retrospective analysis of a distinct clinicohematological entity from a tertiary care center and review of literature.
    Author: Bommannan K, Sachdeva MU, Malhotra P, Kumar N, Sharma P, Naseem S, Ahluwalia J, Das R, Varma N, Prakash G, Khadwal A, Srinivasan R, Varma S.
    Journal: Blood Res; 2016 Mar; 51(1):23-30. PubMed ID: 27104188.
    Abstract:
    BACKGROUND: Plasma cell leukemia (PCL) is a rare and aggressive plasma cell neoplasm. In PCL, clonal plasma cells comprise ≥20% of the peripheral blood (PB) leukocytes and/or the absolute clonal PB plasma cell count is ≥2×10(9)/L. Primary PCL (PPCL) originates de novo, whereas, secondary PCL (SPCL) evolves from pre-existing multiple myeloma. METHODS: Clinicohematological features, immunophenotypic profile, and survival of PCL patients were analyzed retrospectively. RESULTS: Between January 2007 and December 2014, ten PPCL and four SPCL patients were investigated (8 PPCLs and 3 SPCLs had complete clinical data). All were North Indians, sharing common geography and ethnicity. Our cohort showed less frequent renal failure, more frequent hepatomegaly, and non-secretory type disease. In contrast to western literature, flow cytometric immunophenotyping of our cohort revealed altered expression of CD138 (67%), CD56 (33%), and CD20 (0%). With novel therapeutic agents, these PPCL patients had a median overall survival of 15 months. CONCLUSION: We highlight that our PPCL patients from North India had distinct clinicohematological and immunophenotypic profiles. The significance of our findings must be tested in a larger patient cohort and must be supported by molecular and cytogenetic investigations to unmask possible significant effects on pathogenesis.
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