Primary Plasma Cell Leukaemia: Case report and review of the literature

Abstract

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

Figure 1

Peripheral blood smears at x200 magnification showing (A) rouleaux formation, plasma cells (arrows) and (B) atypical lymphocytes (arrowhead).

Figure 2

A: Bone marrow aspirate smear at x1,000 magnification showing atypical plasma cells (arrows). B: Haematoxylin and eosin stain at x400 magnification showing plasma cells.

Figure 3

Immunohistochemistry panel at x400 magnification showing (A) positivity for cluster of differentiation (CD)38, (B) kappa light chain restriction, (C) reduced lambda light chain expression and (D) focal positivity for CD20.