Introduction: Recurrent exacerbations of COPD are commonly referred to the pulmonologist for evaluation and treatment. Immunodeficiency and structural abnormalities should be suspected in patients with frequent exacerbations presenting with increased amounts of purulent sputum, repeated isolation of bacteria (especially encapsulated strains), and the development of new symptoms. Case Presentation: A 72-year-old white male farmer was admitted to the Mayo Clinic Chest hospital service for an acute exacerbation of COPD. He was a former 60-pack-year smoker and had quit in 1970. He complained of dyspnea, a daily productive cough, 6-8 non-bloody stools per day, chronic dermatitis of his palms, and a 50 lb. weight loss over the previous 6 months. In the past two years he was hospitalized for three episodes of Haemophilus influenza pneumonia, and received antibiotics for two episodes of acute bronchitis. Colonoscopic examinations with biopsies revealed indeterminate colitis. Treatment trials of metronidazole did not improve his diarrhea. His past medical history was otherwise non-contributory. His medications included a daily aspirin, albuterol, and atrovent. Physical examination demonstrated bibasilar crackles and 1 mm papules with scaly plaques on both palms. Laboratory tests revealed WBC 5.2 k, Hg 11.9, Plt 357 k, and normal chemistries. Sputum cultures grew Haemophilus influenza. A chest x-ray revealed bilateral infiltrates and fullness in the retrosternal area. Quantitative immunoglobulins revealed hypogammaglobulinemia with IgM 28.1 mg/dl (60-300), IgG 344 mg/dl (700-1500), and normal IgA. PFTs were consistent with severe obstruction. A chest CT demonstrated a 6X4 cm anterior mediastinal mass which was biopsied under CT guidance and was identified as a thymoma. There was no significant bronchiectasis. He received outpatient antibiotic treatment and monthly immunoglobulin replacement. His productive cough, diarrhea and palmer dermatitis dramatically improved. He declined further intervention for the thymoma, such as surgery or radiation therapy. Discussion: Hypogammaglobulinemia, myasthenia gravis, and pure red cell aplasia are the most common parathymic syndromes associated with thymoma. R.A. Good first recognized the association of thymoma with hypogammaglobulinemia in the syndrome which now bears his name. The typical patient with Good's syndrome is in the 5th to 7th decade and develops recurrent bronchitis, sinusitis, or pneumonia and may be complicated by bronchiectasis. Systemic symptoms of weight loss, fatigue, and chronic diarrhea are common. A colitis resembling villous atrophy has been described in some patients which responds to treatment with IVIG, however opportunistic colonic infections need to be excluded. Mucocutaneous candidiasis and lichen planus are also features in some patients. Thymectomy is generally ineffective in improving hypogammaglobulinemia, diarrhea or the skin manifestations. Treatment with IVIG replacement with doses of 200-400 mg/kg is effective and can be given as outpatient therapy every 2-6 weeks depending on trough immunoglobulin levels. Conclusions: Accurate and timely diagnosis of primary immunodeficiency is important to prevent complications which can lead to irreversible tissue destruction. Good's syndrome is the association of thymoma with hypogammaglobulinemia and may be associated with bronchiectasis, weight loss, chronic diarrhea, and dermatitis. Immunoglobulin replacement therapy is central to the long term management of selected patients with hypogammaglobulinemia and may improve many symptoms and prevent complications such as bronchiectasis. Postural drainage and rotating antibiotics have also been used for treating bronchiectasis.
|Original language||English (US)|
|Issue number||4 SUPPL.|
|State||Published - Oct 1 1998|
ASJC Scopus subject areas
- Pulmonary and Respiratory Medicine
- Critical Care and Intensive Care Medicine
- Cardiology and Cardiovascular Medicine