Waldenström’s Macroglobulinemia (WM) is a slow growing hematological malignancy characterized by proliferation of plasma cells, lymphocytes and plasmacytoid lymphocytes in bone marrow secreting monoclonal immunoglobulin IgM and a risk of hyper viscosity syndrome. This case report highlights a unique presentation observed in a 60 years old farmer with neuropathy, and necrotizing soft tissue infection as the primary manifestation of WM. Notably, our patient had recurrent spontaneous hemolysis and presented to us a second time with Acrocyanosis, prompting for evaluation of cold agglutinin disease.This led to the final diagnosis of WM. Treatment was commenced with Bendamustine, Rituximab and Dexamethasone led to complete response (normalization of serum IgM and absence of bone marrow and extramedullary disease). As of the publication of this report, patient is in remission. In our case the patient had manifestations of symmetric peripheral neuropathy which predated all other manifestations, evaluation for the same might have prevented the potentially life-threatening necrotising infection he subsequently presented with.

Keywords:Waldenstorm’s Macroglobulinemia; Cold Agglutinins; Peripheral Neuropathy; Necrotising Soft Tissue Infection; Autoimmune HaemolyticAnaemia; ImmunoglobulinM Monoclonal Gammopathy