Waldenstrom’s Macroglobulinemia - a coincidental finding in a case of carcinoma of buccal mucosa

Mahjabeen Salma,Karimuddin Abdullah Shaik,Nandish Kumar S J,Sudha Madhuri

doi:10.7439/ijbr.v5i1.401

Abstract

Waldenstr's Macroglobulinemia (WM) is a clonal disorder of small lymphocytes that show maturation to plasma cells synthesizing IgM. It is the biological activity of IgM protein that determines most of the clinical manifestations of the disease. WM most closely corresponds to lymphoplasmacytic lymphoma or immunocytoma of the REAL classification of lymphoma. Here, we report a case of a 50 year old male with squamous cell carcinoma of buccal mucosa. On routine investigations we found autoagglutination of RBCs; on workup with this feature we had suspicion of Waldenstr's Macroglobulinemia. The diagnosis of WM was established after cytomorphology and immunohistochemistry (IHC) analysis of the bone marrow which revealed the presence of a lymphoid/lymphoplasmacytoid-like bone marrow infiltrate along with an elevated serum IgM level. Waldenstr's Macroglobulinemia and its co-existence with autoimmune diseases and non hematological malignancies has already been described in the literature, but the present case was asymptomatic for WM with carcinoma of buccal mucosa, which is the first case reported so far.

Highlights

Waldenström's Macroglobulinemia (WM) is a pleomorphic lymphoproliferative disorder characterized by the elevated levels of monoclonal immunoglobulin (IgM) protein secreted by malignant B-cells and paratrabecular lymphoplasmacytic infiltration in the bone marrow
Patients may develop constitutional symptoms, pancytopenia, organomegaly, neuropathy, and symptoms associated with immunoglobulin deposition or hyperviscosity.2,3) WM is incurable with current therapy, and half of the patients die of disease progression; median survival is approximately 5 years.(4) The incidence of development of second cancers was not significantly different between asymptomatic and symptomatic WM and between treated and untreated pati ents.5)
We report a sharp M-band seen in gammaglobulin region detected by protein electrophoresis

Summary

Introduction

WM is a pleomorphic lymphoproliferative disorder characterized by the elevated levels of monoclonal immunoglobulin (IgM) protein secreted by malignant B-cells and paratrabecular lymphoplasmacytic infiltration in the bone marrow. The classical presentation of WM’s patient includes anemia, hepatosplenomegaly, lymphadenopathy and hyperviscosity.1) Infiltration of the bone marrow and extramedullary sites by malignant B-cells and elevated IgM levels account for the symptoms associated with this disease. Patients may develop constitutional symptoms, pancytopenia, organomegaly, neuropathy, and symptoms associated with immunoglobulin deposition or hyperviscosity.2,3) WM is incurable with current therapy, and half of the patients die of disease progression; median survival is approximately 5 years.(4) The incidence of development of second cancers was not significantly different between asymptomatic and symptomatic WM and between treated and untreated pati ents.5)

Case Report

Findings

Discussion