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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
Blood Research ; : 23-30, 2016.
Article in English | WPRIM | ID: wpr-23502
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.
Subject(s)

Full text: Available Index: WPRIM (Western Pacific) Main subject: Plasma / Plasma Cells / Tertiary Healthcare / Leukemia, Plasma Cell / Retrospective Studies / Cohort Studies / Immunophenotyping / Cytogenetics / Renal Insufficiency / Neoplasms, Plasma Cell Type of study: Etiology study / Incidence study / Observational study / Risk factors Limits: Humans Country/Region as subject: Asia Language: English Journal: Blood Research Year: 2016 Type: Article

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Full text: Available Index: WPRIM (Western Pacific) Main subject: Plasma / Plasma Cells / Tertiary Healthcare / Leukemia, Plasma Cell / Retrospective Studies / Cohort Studies / Immunophenotyping / Cytogenetics / Renal Insufficiency / Neoplasms, Plasma Cell Type of study: Etiology study / Incidence study / Observational study / Risk factors Limits: Humans Country/Region as subject: Asia Language: English Journal: Blood Research Year: 2016 Type: Article