Waldenstrom macroglobulinemia presenting as plasma cell leukemia associated with hyperviscosity syndrome

Year 2018, Volume: 5 Issue: 12, 390 - 392, 30.12.2018

Rafiye Çiftçiler , Arzu Sağlam , Elifcan Aladağ İbrahim Celalettin Haznedaroğlu

https://doi.org/10.17546/msd.484778

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.

Keywords

Monoclonal gammopathy, plasma cell leukemia, waldenstrom macroglobulinemia

References

• 1. Gertz MA. Waldenström macroglobulinemia: 2013 update on diagnosis, risk stratification, and management. American journal of hematology. 2013;88(8):703-11.
• 2. Castillo JJ, Olszewski AJ, Cronin AM, Hunter ZR, Treon SP. Survival trends in Waldenström macroglobulinemia: an analysis of the Surveillance, Epidemiology and End Results database. Blood. 2014;123(25):3999-4000.
• 3. Treon SP, Xu L, Yang G, Zhou Y, Liu X, Cao Y, et al. MYD88 L265P somatic mutation in Waldenström's macroglobulinemia. New England Journal of Medicine. 2012;367(9):826-33.
• 4. Varettoni M, Zibellini S, Defrancesco I, Ferretti VV, Rizzo E, Malcovati L, et al. Pattern of somatic mutations in patients with Waldenström macroglobulinemia or IgM monoclonal gammopathy of undetermined significance. haematologica. 2017;102(12):2077-85.
• 5. Treon SP, Ioakimidis L, Soumerai JD, Patterson CJ, Sheehy P, Nelson M, et al. Primary therapy of Waldenström macroglobulinemia with bortezomib, dexamethasone, and rituximab: WMCTG clinical trial 05-180. Journal of Clinical Oncology. 2009;27(23):3830.
• 6. Dao C, Zittoun R, Diebold J, Cadiou M, Reynes M, Gaudric M, et al. Sarcomatous and leukemic forms of Waldenström's macroglobulinemia. La semaine des hopitaux: organe fonde par l'Association d'enseignement medical des hopitaux de Paris. 1975;51(48):2935-42.