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

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

doi:10.5045/br.2016.51.1.23

Quick links

Most Cited Most Read Ahead of Print Archives Go to E-submission

Original Article

Split Viewer

Blood Res 2016; 51(1):

Published online March 31, 2016

https://doi.org/10.5045/br.2016.51.1.23

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

Karthik Bommannan¹, Man Updesh Singh Sachdeva^1*, Pankaj Malhotra², Narender Kumar¹, Prashant Sharma¹, Shano Naseem¹, Jasmina Ahluwalia¹, Reena Das¹, Neelam Varma¹, Gaurav Prakash², Alka Khadwal², Radhika Srinivasan³, and Subhash Varma²

Author Info. +

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

Received: November 21, 2015; Revised: January 28, 2016; Accepted: February 3, 2016

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.

Abstract

Go to

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 ≥2×10⁹/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.

Keywords Clinicohematological profile, North India, Plasma cell leukemia, Immunophenotype, Survival

Article

Original Article

Blood Res 2016; 51(1): 23-30

Published online March 31, 2016 https://doi.org/10.5045/br.2016.51.1.23

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

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

Received: November 21, 2015; Revised: January 28, 2016; Accepted: February 3, 2016

Abstract

Background

Methods

Clinicohematological features, immunophenotypic profile, and survival of PCL patients were analyzed retrospectively.

Results

Conclusion

Keywords: Clinicohematological profile, North India, Plasma cell leukemia, Immunophenotype, Survival

Abstract

Fig 1.

Download

Figure 1.

(A, B) Circulating plasma cells with lymphoplasmacytoid morphology, (C, D) circulating plasma cells with blast like morphology (Magnification ×1,000, May-Grünwald-Giemsa staining).

Blood Research 2016; 51: 23-30https://doi.org/10.5045/br.2016.51.1.23

Table 1 Clinical and hematological parameters of PCL patients.

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..

Table 2 Immunophenotypic profiles of six PPCL cases by multicolor flow cytometry.

Abbreviations: Pos, positive; Neg, negative; Equ, equivocal; Cyto κ, cytoplasmic kappa light chain; Cyto λ, cytoplasmic lambda light chain..

Table 3 Summary of treatment in seven PPCL and two SPCL patients.

^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..

Table 4 Dosage and schedule of the treatment regimen.

^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, per os (by oral); SC, sub-cutaneous; HS, hora somni (bed-time); IV, intra venous..

Table 5 Comparison between the clinical and laboratory data of PPCL cases in the current study and other large case series.

^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..

Table 6 Comparison of marker expression profile with immunophenotyping by flow cytometry performed in six PPCL patients.

Abbreviations: cKappa, cytoplasmic kappa light chain restriction; cLambda, cytoplasmic lambda light chain restriction..