J Med Assoc Thai 2017; 100 (6):706

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Thymoma with Granulomatous Myositis, Pure Red Cell Aplasia, and Hepatosplenomegaly: A Case Report and Review Literature
Korphaisarn K , Apibunyopas T , Hantaweepant C , Sangruchi T , Srinonprasert V , Chiowchanwisawakit P Mail

Background: Thymoma is well-recognized as being associated with various autoimmune diseases. Thymoma associated with granulomatous myositis (GM) is rare and thymoma associated with pure red cell aplasia (PRCA) is somewhat more frequent. However, coexistence of GM and PRCA in a thymoma patient is extremely uncommon.

Case Report: Here, we present the case of a 40-year-old Thai male who presented with progressive proximal muscle weakness with dysphagia, anemia, hepatosplenomegaly, and an anterior mediastinal mass. After a thorough investigation, the patient was diagnosed with stage I type AB thymoma with GM and PRCA. Following treatment with thymectomy, corticosteroids, and immunosuppressive agents, muscle weakness improved, hepatosplenomegaly resolved, and laboratory abnormalities normalized. The patient was then able to resume his normal daily activities and return to work.

Conclusion: In this case, we report a rare association of GM, PRCA, hepatosplenomegaly, and thymoma. Early detection with prompt treatment with tumor removal and immunosuppressive medications resulted in a good clinical response. Based on our review of the literature, this is the first published case report to describe this combination of coexisting conditions.

Keywords: Thymoma, Granulomatous myositis, Pure red cell aplasia, Hepatosplenomegaly, Paraneoplastic syndrome


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