A case of Good's syndrome / 천식및알레르기

ABSTRACT

A 52-year-old woman suffering from recurrent orogenital ulcerations with superficial candidiasis and chronic mucocutaneous herpes simplex infection was admitted for headache and productive cough. She had undergone a thymectomy due to thymoma of an epithelial cell type several years ago. Radiologic and immunologic examinations revealed bronchiectasis in the left lower lobe, severe deficiency in all isotypes of immunoglobulin and an abnormal delayed hypersensitivity to ubiquitous antigens on skin test. Analysis of lymphocyte subsets in peripheral blood and bone marrow showed marked decreases in the proportion of cells bearing B cell markers. Her symptoms and signs were improved with antibiotics, antifungal and antiviral agents, and monthly administration of high dose intravenous immunoglobulin (IVIG, 400mg/kg). This is a rare case of Good's syndrome presenting, a humoral and cellular immune deficiency syndrome related with thymoma. The pathogenic mechanism of hypogammaglobulinemia in this case might be caused by a block in the early stage of B cell differentiation.