Abstract
Waldenström's macroglobulinemia is a B-cell neoplasm characterized by infiltration of the bone marrow by a lymphoplasmacytic infiltrate and an IgM monoclonal gammopathy. It is an uncommon disease with overall incidence of approximately 3 per million persons per year, accounting for approximately 1% to 2% of all hematologic cancers. It has only one-sixth the estimated prevalence of plasma cell myeloma. Disease symptoms can be due to infiltration of bone marrow and other tissue sites by malignant lymphoplasmacytic cells or due to the effects of elevated serum IgM levels. However, patients may present with constitutional symptoms only or may be asymptomatic. In our case, patient presented with chief complaints of fatigability and dyspnoea and was misdiagnosed as plasma cell leukemia on peripheral blood film and bone marrow morphology, but turned out to be a case of Waldenström's macroglobulinemia on cytoflorometry. The patient was referred for chemotherapy but expired on 10th day of admission. The suspected cause of death was cardiorespiratory failure.
Highlights
Waldenstrom’s macroglobulinemia (WM) is a pleomorphic lymphoproliferative disorder characterized by production of a monoclonal immunoglobulin (IgM) protein and a lymphoplasmacytic infiltrate in the bone marrow
Since there was no history of joint pains and lytic bone lesions, hypercalcemia was not observed, and due to considerable overlap of clinical and morphological features in various B cell lymphoproliferative disorders [1], the sample was sent for serum electrophoresis, flow cytometry, and other investigations
If this would be the case of primary PCL or WM terminating into PCL, the flow cytometry of cells must show a population that is deficient in B- and T-cell-specific markers such as CD10, CD20, CD5, CD45, and sIgM, but strongly expressing CD38, CD138, and cytoplasmic lambda light chains, while this case was found to express pan B-cell markers, typical of Waldenstrom’s macroglobulinemia
Summary
Waldenstrom’s macroglobulinemia (WM) is a pleomorphic lymphoproliferative disorder characterized by production of a monoclonal immunoglobulin (IgM) protein and a lymphoplasmacytic infiltrate in the bone marrow. The median age at presentation is 63 years [1]. The most consistent feature of the bone marrow or lymph node of patients with WM is the presence of pleomorphic B-lineage cells at different stages of maturation, such as small lymphocytes, lymphoplasmacytoid cells, and plasma cells [2]. The symptoms and signs are a function of lymphocytic infiltration of marrow leading to cytopenias, especially anemia (commonly manifesting as fatigue), infiltration of peripheral tissues leading to lymphadenopathy and hepatosplenomegaly in 20–30% of patients, and consequences of IgM in the circulation and deposited in organs. The median age of survival ranges between 5 and 10 years
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