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.
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
Similar
MEDLINE
...
LILACS
LIS