Waldenstrom macroglobulinemia presenting as plasma cell leukemia associated with hyperviscosity syndrome

Abstract

Objective: Waldenstrom macroglobulinemia (WM) is a rare indolent neoplastic disease characterized by a wide range of clinical presentations related to the direct tumor infiltration. The disease is characterized by monoclonal immunoglobulin M protein in the serum and infiltration of bone marrow with lymphoplasmacytic cells. Case report: We, herein, present an unusual case of WM. A 77-year-old woman admitted to the hospital with fatigue, anorexia, and fever. She had white blood cell elevation and splenomegaly. The patient had no peripheral lymphadenopathy. A large number of plasmablast-like cells were seen in the peripheral blood smear. Laboratory studies revealed a white blood cell count of 54.8 × 103/µl, hemoglobin level of 8.2 g/dl and platelet count was 120 × 103/µl. The diagnosis of WM was established after immunohistochemical analysis of the patient's bone marrow that revealed the presence of a lymphoid/lymphoplasmacytoid-like bone marrow infiltrate along with an elevated serum IgM level. The patient responded to the chemotherapy both clinically and serologically. This case is unusual since numerous plasmablast like cells were seen in peripheral blood smear like plasma cell leukemia at the admission to the hospital. Conclusion : This is the case report of a patient with Waldenstrom macroglobulinemia presenting like plasma cell leukemia in the first admission adding to the spectrum of clinical presentations seen in this disease. This adds to the wide variety of clinical presentations of Waldenstrom macroglobulinemia.