Immunological disturbances in Good's syndrome.
BACKGROUND
Immunodeficiency with a thymoma (Good's syndrome) is a rare condition occurring in patients with adult-onset hypogammaglobulinaemia.


METHODS
We describe the case of a 38-yr-old woman with an upper mediastinal mass and inflammatory infiltrations in the lungs. After thymectomy, the patient's condition did not improve. The HRCT scan showed bronchiectasies with parenchymal opacities. As pulmonary infection persisted despite wide spectrum antibiotic therapy, additional tests were performed to diagnose an immunodeficiency. Serum immunoglobulin levels were very low. T cell response to mitogens was normal, but to Staphylococcus aureus Cowan I was impaired. Immunophenotyping of peripheral blood and bone marrow aspirate showed a very low number of B-cell at all the stages of development (CD10+CD19+, CD5+CD20). In peripheral blood 2.5% of CD19+ lymphocytes were found. On the basis of clinical history and immunological analysis, Good's syndrome was recognized. Treatment with intravenous gammaglobulin and antibiotics improved the patient's performance.


CONCLUSIONS
Measurement of serum immunoglobulin concentration is recommended for all patients suspected of thymoma.
Fijolek
Justyna
J
Institute of Tuberculosis and Lung Diseases, Warsaw, Poland.
Wiatr
Elzbieta
E
Demkow
Urszula
U
Orlowsk
Tadeusz Maria
TM
D000328:Adult; D000361:Agammaglobulinemia / Q000097:blood / Q000150:complications / Q000276:immunology; D001402:B-Lymphocytes / Q000276:immunology; D005260:Female; D006801:Humans; D007136:Immunoglobulins / Q000097:blood; D007153:Immunologic Deficiency Syndromes / Q000097:blood / Q000150:complications* / Q000276:immunology*; D016130:Immunophenotyping; D013945:Thymoma / Q000097:blood / Q000150:complications* / Q000276:immunology*
D007136:Immunoglobulins