Blood Res 2016; 51(1):
Published online March 31, 2016
https://doi.org/10.5045/br.2016.51.1.23
© The Korean Society of Hematology
1Department of Hematolog, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
2Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
3Department of Cytopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Correspondence to : Correspondence to Man Updesh Singh Sachdeva, M.D. Department of Hematology, Postgraduate Institute of Medical Education and Research, Kairon Block, Sector 12, Chandigarh 160012, India. drmanupdeshpgi@yahoo.co.in
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
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
Clinicohematological features, immunophenotypic profile, and survival of PCL patients were analyzed retrospectively.
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.
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.
Keywords Clinicohematological profile, North India, Plasma cell leukemia, Immunophenotype, Survival
Blood Res 2016; 51(1): 23-30
Published online March 31, 2016 https://doi.org/10.5045/br.2016.51.1.23
Copyright © The Korean Society of Hematology.
Karthik Bommannan1, Man Updesh Singh Sachdeva1*, Pankaj Malhotra2, Narender Kumar1, Prashant Sharma1, Shano Naseem1, Jasmina Ahluwalia1, Reena Das1, Neelam Varma1, Gaurav Prakash2, Alka Khadwal2, Radhika Srinivasan3, and Subhash Varma2
1Department of Hematolog, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
2Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
3Department of Cytopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Correspondence to: Correspondence to Man Updesh Singh Sachdeva, M.D. Department of Hematology, Postgraduate Institute of Medical Education and Research, Kairon Block, Sector 12, Chandigarh 160012, India. drmanupdeshpgi@yahoo.co.in
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
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
Clinicohematological features, immunophenotypic profile, and survival of PCL patients were analyzed retrospectively.
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.
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.
Keywords: Clinicohematological profile, North India, Plasma cell leukemia, Immunophenotype, Survival
(
Note: Age and gender were available for ten PPCLs and four SPCLs. Clinicohematological and biochemical data were available for eight PPCLs and three SPCLs. SPEP and UPEP data were available for six PPCLs and three SPCLs. Serum protein immunofixation data was available for six PPCLs and two SPCLs..
Abbreviations: PPCL, primary plasma cell leukemia; BM, bone marrow; B2M, beta-2-microglobulin; κ, kappa light chain; λ, lambda light chain; MM, multiple myeloma; PB, peripheral blood; PEP, protein electrophoresis; S. Ca, serum calcium; S. Cr, serum creatinine; TLC, total leukocyte count; NA, not applicable..
Abbreviations: Pos, positive; Neg, negative; Equ, equivocal; Cyto κ, cytoplasmic kappa light chain; Cyto λ, cytoplasmic lambda light chain..
a)Not affordable for bortezomib..
Note: Among ten PPCLs, one lost follow-up before start of therapy and two opted against therapy. Out of four SPCLs, two opted against therapy..
Abbreviations: PPCL, primary plasma cell leukemia; SPCL, secondary plasma cell leukemia; CR, complete response; PR, partial response; sCR, stringent CR..
a)Started with 100 mg, maximum 200 mg. Dose modifications: In case of peripheral neuropathy, thalidomide dose was reduced by 50% and bortezomib was reduced to 1 mg/m2. In case of renal impairment, melphalan dose was reduced by 25% if Cr clearance was <30 mL/min. For lenolidomide, the dose adjustments were: 10 mg for Cr clearance of <30 mL/min, 15 mg for Cr clearance <30–60 mL/min and 5 mg for patients on hemodialysis. The treatment response criteria was as defined by IMWG uniform response criteria [22]..
Abbreviations: PO,
a)Median value, b)Median not available, c)98% had ≥40% PC, d)≥2 mg/dL in 44%, e)≥6 mg/mL in 65%..
Abbreviations: κ, kappa light chain; λ, lambda light chain; NA, Not available; PC, plasma cells; PB, peripheral blood; BM, bone marrow; B2M, beta-2-microglobulin; Hb, hemoglobin; S.Cr, serum creatinine; S.Ca, serum calcium..
Abbreviations: cKappa, cytoplasmic kappa light chain restriction; cLambda, cytoplasmic lambda light chain restriction..
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