Source: Annual Congress 2012 - The good clinical practice: useful case report
Disease area: Respiratory infections

Abstract

A 62-year-old woman with a history of thymectomy for capsule-invasion-free, type AB thymoma, presented with cough, dyspnea and fever. She had frequent respiratory tract infections for 9 months. Her initial physical examination was normal. Abnormal laboratory studies on admission were anemia,leukocytosis, proteinuria, elevated erithrocyte sedimentation rate, C-reactive protein and procalcitonin levels. On chest tomography, multiple mediastinal and hiler lymph nodes, bilateral traction bronchiectasis and milimetric nodules were seen. E.Coli was isolated in bronchoalveolar lavage. TBNA and TBB were negative for probable thymoma metastasis.
For the probability of Good‘s Syndrome, serum immunoglobulin levels were measured and found normal for IgA, low for IgG and high for IgM. Flow cytometry demonstrated 8% of CD19+, 12% of CD56+, 14% of CD57+, 30% of CD4+, 37% of CD8+ cells in total lymphocytes; consistent with B-cell immunodeficiency. With the diagnosis of Good‘s Syndrome, intravenous immunoglobulin treatment was started. During her follow-up,she was hospitalized twice for respiratory tract infections. Now she is on ciprofloxacin profilaxis.
Good‘s syndrome is a rare cause of combined B or T cell deficiency. Its main characteristics are hypogammaglobulinemia, reduced or absent B cells, reduced serum levels of IgG, IgA and IgM,recurrent sinopulmonary infections caused by encapsulated microorganisms. Management of syndrome includes surgical resection of thymoma, treatment of infections and immunoglobulin replacement therapy.
Good Syndrome is a rare but treatable condition and it should be considered in patients with the diagnosis of thymoma and frequent respiratory tract infections.

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Citations should be made in the following way:
M. Türk, N. Köktürk, N. Ekim (Ankara, Turkey). Hypogammaglobulinemia, reduced B cell count and recurrent sinopulmonary infections: Good‘s syndrome. Eur Respir J 2012; 40: Suppl. 56, 3518

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