Plasma cell leukemia in North India: retrospective analysis of a distinct clinicohematological entity from a tertiary care center and review of literature.
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.


CONCLUSIONS
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.
Department of Hematolog, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Karthik
K
Bommannan
Department of Hematolog, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Man Updesh Singh
MU
Sachdeva
Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Pankaj
P
Malhotra
Department of Hematolog, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Narender
N
Kumar
Department of Hematolog, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Prashant
P
Sharma
Department of Hematolog, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Shano
S
Naseem
Department of Hematolog, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Jasmina
J
Ahluwalia
Department of Hematolog, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Reena
R
Das
Department of Hematolog, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Neelam
N
Varma
Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Gaurav
G
Prakash
Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Alka
A
Khadwal
Department of Cytopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Radhika
R
Srinivasan
Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Subhash
S
Varma
Clinicohematological profile; Immunophenotype; North India; Plasma cell leukemia; Survival