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Thymoma with immunodeficiency/Good syndrome associated with myasthenia gravis
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- Takai Shunsuke
- Departments of Neurology, International University of Health and Welfare Hospital, Tochigi, Japan
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- Tagawa Asako
- Departments of Neurology, International University of Health and Welfare Hospital, Tochigi, Japan
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- Ogawa Tomoko
- Departments of Neurology, International University of Health and Welfare Hospital, Tochigi, Japan
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- Kato Hiroyuki
- Departments of Neurology, International University of Health and Welfare Hospital, Tochigi, Japan
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- Saito Noriko
- Departments of Thoracic Surgery, International University of Health and Welfare Hospital, Tochigi, Japan
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- Okada Shinya
- Departments of Pathology, International University of Health and Welfare Hospital, Tochigi, Japan
Bibliographic Information
- Other Title
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- 胸腺腫関連重症筋無力症の経過中に免疫不全（Good症候群）を合併した1例
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Description
<p>Good syndrome is a rare condition in which thymoma is associated with hypogammaglobulinemia; it is characterized by repeated respiratory or systemic infections caused by bacteria, viruses, and fungi, as well as with various autoimmune disorders such as pure red cell aplasia. A 65-year-old woman was admitted to our hospital with ptosis and abdominal muscle weakness. Based on the presence of anti-acetylcholine receptor (Ach-R) antibodies, she was diagnosed with myasthenia gravis (MG). At that time, invasive thymoma of Masaoka stage IVa was also detected. Regression of thymoma and clinical remission of MG was achieved by chemotherapy followed by high-dose corticosteroid. However, several months later, the patient started developing repeated bacterial respiratory tract infections, cytomegalovirus infections, and esophageal and systemic candidiasis. Laboratory tests revealed a marked decrease of serum gamma-globulin levels (IgG 586 mg/dl, IgA 32 mg/dl, IgM 29 mg/dl) and severe reduction in the B cells ratio, as well as a decrease in the CD4+CD25+T cell to CD4+CD25−T cell ratio indicative of deregulation of CD4+T cell activation. These results suggested that the patient impaired humoral and cell-mediated immune responses. We continued the treatment with antibiotics and regular immunoglobulin supplementation through intravenous injections. Although autoimmune disorders are often observed in Good syndrome, the association with MG is quite rare. The case report is followed by the review of literature.</p>
Journal
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- Rinsho Shinkeigaku
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Rinsho Shinkeigaku 57 (5), 208-213, 2017
Societas Neurologica Japonica
