内科: オープンアクセス
オープンアクセス

概要

Good's Syndrome: A Case with Recurrent Respiratory Infections and a Brief Review of the Literature

Sibel Ersan, Gursel Ersan, Alper Toker, Cagatay Arslan, Sabri Atalay and Sukran Kose

Background: Thymomas are tumors associated with autoimmune disorders like myasthenia gravis, Pure Red Cell Aplasia (PRCA), and acquired hypogammaglobulinemia (Good’s syndrome). The failure of thymomas to express autoimmune regulator has been shown to be a potential contributing factor to autoimmunity.

Case report: A 78-year old man with recurrent sinopulmonary infections, and megaloblastic anemia diagnosed PRCA by bone marrow aspiration. Further investigation revealed thymoma associated acquired hypogammaglobulinemia. Thymectomy failed to resolve PRCA, and the patient was commenced on periodic Intravenous Immunglobulin (IVIG) therapy for both to achieve improvement of PRCA and to support humoral immunity. On IVIG therapy the frequency of respiratory infections decreased significantly. The patient was also given prophylaxis against herpes viruses, and Pneumocystis jiroveci, and got vaccination for capsulated bacteria. As the trial of IVIG therapy had no effect on PRCA he was started oral corticosteroid treatment. PRCA resolved and steroid therapy was tapered gradually. He has been on remission for nine years without requirement of systemic steroid. The case is a very rare kind of it’s in the literature.

Conclusion: The clinical experience on management of Good’s syndrome and PRCA by steroid therapy and regular IVIG infusions, and also the use of prophylactic agents and immunization against opportunistic infections was underlined here. The careful monitoring for any signs of oncoming respiratory infection and early intervention has utmost importance for this special group of patients.