Severe Thrombocytopenia in a Splenectomized Patient with Waldenstrom Macroglobulinemia

Vinay Minocha^1, , Dat Pham¹, Megan Brown², Arun Gopinath¹, Madhu Emani¹ and Lara Zuberi¹

¹Division of Medical Oncology, Department of Internal Medicine, University of Florida, Jacksonville, FL, USA

²Department of Pathology, University of Florida, Jacksonville, FL, USA

Cite this paper:
Vinay Minocha, Dat Pham, Megan Brown, Arun Gopinath, Madhu Emani and Lara Zuberi. Severe Thrombocytopenia in a Splenectomized Patient with Waldenstrom Macroglobulinemia. American Journal of Medical Case Reports. 2016; 4(8):275-278. doi: 10.12691/ajmcr-4-8-7

Abstract

Introduction. Patients with Waldenström macroglobulinemia (WM) can present with a diverse array of findings. One such manifestation is autoimmune thrombocytopenia which may occur as a consequence of autoantibody activity against platelets. Case Presentation. We report a case of a 57-year- old African American male who presented with a two-week history of fatigue, recurrent epistaxes and gingival hemorrhage. His past medical history was significant for liver cirrhosis secondary to Hepatitis C and a splenectomy 2 years prior for traumatic rupture of the spleen. Complete blood count demonstrated platelets 3 x10⁹/l. Serum protein electrophoresis and immunofixation demonstrated an IgM Lambda monoclonal paraprotein of 7.6 g/dl. Serum viscosity was elevated at 10.8 cP. He was diagnosed with a hyperviscosity syndrome secondary to suspected Waldenstrom’s Macroglobulinemia (WM) and plasmapheresis was emergently initiated due to bilateral retinal vein thrombosis. A bone marrow aspirate and biopsy revealed an interstitial plasmacytoid proliferation which was CD138 positive and lambda restricted. With plasmapheresis, his serum IgM was lowered to less than 4 g/dl and his serum viscosity decreased to 4.4 cP. A weekly regimen of bortezomib, dexamethasone and rituximab (BDR) was used for treatment. On follow-up three months later he continued on the weekly BDR regimen and his platelet count had normalized to 219 x10⁹/l. Conclusion. Severe thrombocytopenia is an uncommon presentation of WM that may be secondary to complement mediated thrombocytopenia associated with monoclonal IgM antiplatelet antibody. In such cases, therapies for ITP such as corticosteroids and high-dose immunoglobulins may not be as effective as anti-tumor chemotherapy.

Keywords:
Waldenström macroglobulinemia Thrombocytopenia immune thrombocytopenic purpura (ITP) hyperviscosity syndrome splenectomy hemorrhage complement mediated thrombocytopenia

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