Primary plasma cell leukemia: A report of two cases of a rare and aggressive variant of plasma cell myeloma with the review of literature.
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Date
2016-10
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Abstract
Plasma cell leukemia (PCL) is a rare and aggressive variant of myeloma
accounting for 2-3% of all plasma cell dyscrasias characterized by the presence
of circulating plasma cells. The diagnosis is based on the % (≥20%) and absolute
number (≥2x109/L) of plasma cells in the peripheral blood. The incidence of
primary PCL (pPCL) is very rare and reported to occur in <1 in a million. It is
classified as either pPCL occurring at diagnosis or as secondary PCL in patients
with relapsed/refractory myeloma. pPCL is a distinct clinicopathological entity with
different cytogenetic and molecular findings. The clinical course is aggressive with
short remissions and survival duration. We report two cases of pPCL, both having
acute onset of illness, varied clinical presentation with one of them showing “hairy
cell morphology,” with rapidly progressing renal failure, and was not suspected
to be plasma cell dyscrasia clinically. A detailed hematopathological evaluation
clinched the diagnosis in this case. It is recommended that techniques such
as immunophenotyping by flow cytometry and protein electrophoresis must
be performed for confirmatory diagnosis. A detailed report of two cases and a
review of PCL are presented here.
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Keywords
Immunophenotyping, plasma cell dyscrasias, plasma cell leukemia
Citation
Gangadhar Prithal, Ahmed Zulfikar, Pai Muktha R, Sandhya I. Primary plasma cell leukemia: A report of two cases of a rare and aggressive variant of plasma cell myeloma with the review of literature. Indian Journal of Pathology & Microbiology. 2016 Oct-Dec 59(4): 541-544.