Plasma cell leukemia is a rare and aggressive variant of
myeloma characterized by the presence of circulating plasma cells greater than
2x109/l in the peripheral blood. It is classified as primary if present at
diagnosis, or secondary, if evolved from relapsed or refractory multiple
myeloma. It has an aggressive clinical course with limited durations of
remission and survival. It may at times, present as a lymphoproliferative
disorder, leading to misdiagnosis if proper diagnostic tools are not used. This
in turn, will lead to adoption of a completely different line of treatment. Our
patient presented with a disturbed dietary regimen and significant weight loss.
He was a known case of hypothyroidism since 2009 for which he was on
medications. Before landing at our centre, complete blood counts were done at
another centre, which showed bicytopenia with hyperleucocytosis. Bone marrow
biopsy made a diagnosis of lymphoproliferative disorder while morphologically a
provisional diagnosis of Hairy cell leukemia was made. At our center, complete
blood count was repeated, which again showed similar counts with bicytopenia
and leucocytosis, but the morphological examination of his peripheral smear
showed the presence of 80% plasma cells. Immunophenotyping showed CD23(+) and
CD56(+) while the immunohistochemistry showed CD23(+), CD56(+) and CD138(+).
Cytogenetics showed the presence of t(4;14) in 5% of the cells examined. Based
on the above mentioned investigations, a diagnosis of Plasma cell leukemia was
reached and the patient was placed on a treatment regimen including
Lenalidomide, Bortezumib and Dexamethasone. His blood counts reached the normal
range within a week of starting the treatment. This regimen was given for a
full months and there was an overall better response. His last follow up was a
month ago and he is doing well till date. To conclude, in the wake of
constantly advancing diagnostic approaches and availability of more precise
investigative tools, one should not hesitate in employing these to reach a more
accurate diagnosis as is clinically and economically possible; so that the
patient may receive the most appropriate treatment.
Author(s) Details
Bushra Kaleem Author(s) Details
Department of Haematology, The Indus Hospital, Karachi, Pakistan.
View Book :- http://bp.bookpi.org/index.php/bpi/catalog/book/212
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