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A Tale of Primary Plasma Cell Leukemia in Young

A Tale of Primary Plasma Cell Leukemia in Young
#00065325
Author: Nandhini Gangadaran; Ruchi Gupta
Category: Lymphoma: Mature B-cell and Plasma cell Neoplasms > Plasma Cell Neoplasm > Plasma cell myeloma
Published Date: 09/23/2024

A 23-year-old woman presented with intermittent fever for one month. Physical examination was notable for massive splenomegaly, absence of hepatomegaly and lymphadenopathy. Hematological evaluation revealed severe anemia (30g/L), thrombocytopenia (21x109/L) and leucocytosis, 61.96 × 109/L. Serum urea, S. creatinine and LDH levels were deranged. Peripheral smear and bone marrow examination showed 40% medium sized bilobed binucleate cells with moderate basophilic agranular cytoplasm, morphologically mimicking hypogranular promyelocytes or monocytoid blasts (Panel A- Peripheral blood smear, Leishman stain; Panel B- Bone marrow aspirate, MGG stain; 100x objective). On flow cytometry, these atypical cells were negative for CD45, HLA-DR, CD34, TdT, MPO, CD13, CD117, CD7, CD20, CD19, CD27, CD81, CD56 however, brightly expressed CD38, CD138, CD319 with kappa restriction, confirming to the phenotype of abnormal clonal plasma cells (Panel C- bright-pink, plasma cells). SPE and IFE, performed later showed IgG kappa type of monoclonal protein, two bands, 0.3 and 1g/dl (Panel D). A diagnosis of primary plasma cell leukemia (PCL) was rendered. The patient deteriorated rapidly and succumbed within 48 hours of diagnosis.

This case highlights the decoy nature of the plasma cells and unusual aggressive clinical course of PCL in young individuals. Awareness of such morphological variants is essential for early intervention.