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Transcript
‘’Hairy’’ Plasma cells
A 68 years male presented with history of weakness, body aches and weight loss.
Physical examination revealed anemia and hepatomegaly. Baseline peripheral blood
counts showed hemoglobin 10.7 G/DL, hematocrit 37.2%, mean corpuscular volume
103.9 FL, mean corpuscular hemoglobin 34.5 PG, white blood cells 86.2 x 109/L,
absolute neutrophil count 2.5 x 109/L and platelets 74 x 109/L. Subsequently bone
marrow was done for further workup.
His peripheral blood film showed 60% lymphoplasmacytoid cells with hairy cytoplasmic
projections (panel A & B). Based on peripheral blood film findings, initial diagnosis was
of Hairy cell leukemia. Bone marrow aspirate interestingly showed infiltration with
plasma cells that exhibited pleomorphic features and cytoplasmic projections (panel C &
D). Bone trephine biopsy was performed which showed interstitial infiltration with
plasma cells and decrease haematopoietic precursors (panel E). These plasma cells
were positive for CD 138 immunohistochemical stain (panel F). Flowcytometric
immunophenotyping was also performed and the gated cell population showed bright
positivity for CD 138, CD 56 & CD 38 that are expressed by plasma cells and this
population was negative for myeloid markers like CD 13, CD 33 & CD 117 and lymphoid
markers like CD3, CD 5, CD 7, CD 10, CD 11c, CD 19, CD 20, CD 22, CD 23, CD 25 &
CD 103 along with cCD79a while population was also negative for CD 34, TdT. Hence
the diagnosis of Plasma cell leukemia (PCL) was made.
PCL is a scarce and belligerent haematological malignancy. Patients with PCL have a
very poor prognosis with median survival is measured in months (1). There is presence
of more than 20% plasma cells on peripheral blood film, however morphology can
sometimes be tricky (like the above mentioned case) and may lead to wrong diagnosis
and delayed treatment. Therefore it is always important to confirm the probable
diagnosis with additional testing.
Figure1
Keywords:
Plasma cell dyscrasia , Plasma cell leukemia , Hairy Plasma cells
Authorship Contributions
Concept: Natasha Bahadur Ali,
Design: Ayesha Majeed Memon, Data
Collection or Processing: Ayesha Majeed Memon,
Analysis or Interpretation: Natasha Bahadur Ali,
Literature Search: Ayesha Majeed Memon,
Writing: Natasha Bahadur Ali and Ayesha Majeed Memon
.
Conflict of Interest:
The authors of this paper have no conflicts
of interest, including specific financial interests, relationships,
and/or affiliations relevant to the subject matter or materials
included.
Reference:
(1) R.A. Kyle, J.E. Maldonado, E.D. Bayrd. Plasma cell leukemia. Report on 17
cases. Arch Intern Med. 1974;133:813-818