Plasma cell leukemia in North India: retrospective analysis of a distinct clinicohematological entity from a tertiary care center and review of literature.
- Author:
Karthik BOMMANNAN
1
;
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
Author Information
- Publication Type:Original Article
- Keywords: Clinicohematological profile; North India; Plasma cell leukemia; Immunophenotype; Survival
- MeSH: Cohort Studies; Cytogenetics; Geography; Hepatomegaly; Humans; Immunophenotyping; India*; Leukemia, Plasma Cell*; Leukocytes; Multiple Myeloma; Neoplasms, Plasma Cell; Plasma Cells*; Plasma*; Renal Insufficiency; Retrospective Studies*; Tertiary Care Centers*; Tertiary Healthcare*
- From:Blood Research 2016;51(1):23-30
- CountryRepublic of Korea
- Language:English
- 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.
