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Plasma cell leukaemia (PCL) is one of the most aggressive and rarest forms of plasma cell dyscrasia. However, the diagnostic criteria for this condition have not yet been revised and there is no specific treatment to significantly improve the course of the disease. We report a 69-year-old male who presented to the Lok Nayak Hospital, New Delhi, India, in 2017 with dyspnoea and chest pain. A peripheral blood smear showed an absolute plasma cell count of 2.16 × 109/L. A bone marrow examination showed 61% atypical plasma cells exhibiting kappa light chain restriction. Biochemical investigations were consistent with a diagnosis of primary PCL with renal involvement. Bortezomib-based chemotherapy was initiated, which resulted in an improvement in the patient’s haematological and biochemical parameters. This case report includes a comprehensive review of the clinical and diagnostic features, pathobiology and treatment of PCL.

Keywords: Plasma Cell Leukemia; Multiple Myeloma; Plasma Cells; Case Report; India.

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How to Cite
Singh, S., Rath, A., & Yadav, S. (2018). Primary Plasma Cell Leukaemia: Case report and review of the literature. Sultan Qaboos University Medical Journal, 18(3), e397–401.