A Diagnostic Dilemma: Waldenström's Macroglobulinemia/Plasma Cell Leukemia

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.

A male with longstanding anaemia was diagnosed with Waldenström’s Macroglobulinaemia: A case report

“…Infiltration of peripheral tissues, leading to lymphadenopathy and hepatosplenomegaly, occurs in 20-30% of patients. The consequences of IgM in the circulation manifest as symptoms of hyper viscosity, mainly neurological, which includes blurring of vision, headache, and rarely stroke and coma [7]. The association of macroglobulinaemia with neurological symptoms such as polyneuropathy has been referred to as the Bing-Neel syndrome [8. Attempts to better describe Waldenström macroglobulinemia have been made recently by both the WHO Lymphoma Classification, a consensus group formed at the Second International Workshop on Waldenström Macroglobulinaemia and the Mayo Clinic [5].The consensus group formed restricts the diagnosis exclusively to cases with lymphoplasmacytic lymphoma and an IgM monoclonal protein and it eliminates the requirement for either a minimum amount of marrow involvement by lymphoplasmacytic lymphoma or a threshold concentration of IgM in the serum.…”

“…It was recommended that patients with IgM mono clonal gammopathy of undetermined significance (MGUS) and smoldering Waldenström macroglobulinemia be observed without treatment and be evaluated every six months [5]. The median period of survival ranges between 5 and 10 years [7].…”