Plasma Cell Leukemia: A Review of 3 Cases Managed in Kenya

Abstract

Plasma Cell Leukemia (PCL) is a rare and aggressive form of plasma cell dyscrasia that can arise either de novo (primary plasma cell leukemia) or evolve from previously diagnosed and treated multiple myeloma (secondary PCL). We highlight three clinical cases with very different presentations as a reminder of this diagnosis. The cases also highlight the diversity and variability that cover a patient’s journey that is highly dependent on accessibility based on financial capability and social support. The clinical presentation is more aggressive due to the higher tumour burden and more proliferative tumor cells with cytopenias being profound and more organomegaly. The diagnosis is made based on at least 20% of total white blood cells being circulating plasma cells with a peripheral blood absolute plasma cell count of at least 2 × 109/l. Treatment with novel agents followed by autologous stem cell transplant in those who are transplant eligible leads to better outcomes.

Plasma Cell Leukemia: An Aggressive Disease, a Cause of Diagnostic Error

“…On physical examination, she presented with hepatomegaly, bone marrow failure syndrome and obtundiness. Other patients may present with pleural effusion, neurological deficits due to CNS involvement, or palpable extramedullary soft tissue plasmacytomas (8) .…”