Waldenstrom Macroglobulinemia / Plasma Cell Leukemia: A Diagnostic Dilemma

Bhawna Sethi; Kalam Singh Butola; Yogesh Kumar

Bhawna Sethi Department of Pathology, S.H.K.M. Government Medical College, Nuh, 122107, Haryana, India.
Kalam Singh Butola Department of Medicine, Veer Chandra Singh Garhwali, Government Medical Sciences and Research Institute, Srinagar, Uttarakhand, India.
Yogesh Kumar Department of Ophthalmology, S.H.K.M. Government Medical College, Nuh, 122107, Haryana, India.

Keywords: B-cell lymphoproliferative disorder, lymphoplasmacytic lymphoma, monoclonal gammopathy, plasma cell dyscrasias, mature B cell neoplasm

Abstract

Waldenstrom macroglobulinemia (WM) is a subset of lymphoplasmacytic lymphoma and is categorized under mature B-cell lymphoid neoplasm. This lymphoproliferative disorder is characterized by infiltration of the bone marrow by a clonal lymphoplasmacytic infiltrate (?10%) and an IgM monoclonal gammopathy. WM infiltrate comprises Blineage cells with predominance of small lymphocytes, admixed with lymphoplasmacytoid cells, plasma cells and often increased mast cells. This similar spectrum of cells, which is present in the bone marrow, may be present in the peripheral blood as well. With marked plasmacytic differentiation, it may be challenging to differentiate it from plasma cell leukemia. However, this differentiation is crucial for prognostication and therapeutic decision-making. We present a case that manifested with fatigability and dyspnoea, and was misdiagnosed as plasma cell leukemia on peripheral blood film and bone marrow morphology. It finally turned out to be a case of Waldenstrom macroglobulinemia on cytoflorometry, highlighting the role of elaborated workup and ancillary techniques to direct the accurate diagnosis and management.