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KMID : 0366220160510010023
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Korean Journal of Hematology
2016 Volume.51 No. 1 p.23 ~ p.30
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Plasma cell leukemia in North India: retrospective analysis of a distinct clinicohematological entity from a tertiary care center and review of literature
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Karthik Bommannan
Man Updesh Singh Sachdeva
Pankaj Malhotra
Narender Kumar
Prashant Sharma
Shano Naseem
Jasmina Ahluwalia
Reena Das
Neelam Varma
Gaurav Prakash
Alka Khadwal
Radhika Srinivasan
Subhash Varma
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Abstract
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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 ≥2×109/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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KEYWORD
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Clinicohematological profile, North India, Plasma cell leukemia, Immunophenotype, Survival
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