Immunological disturbances in Good's syndrome.
Clin Invest Med
; 32(4): E301-6, 2009 Aug 01.
Article
em En
| MEDLINE
| ID: mdl-19640334
ABSTRACT
BACKGROUND:
Immunodeficiency with a thymoma (Good's syndrome) is a rare condition occurring in patients with adult-onset hypogammaglobulinaemia. CLINICAL REPORT 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.CONCLUSION:
Measurement of serum immunoglobulin concentration is recommended for all patients suspected of thymoma.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Timoma
/
Síndromes de Imunodeficiência
Limite:
Adult
/
Female
/
Humans
Idioma:
En
Revista:
Clin Invest Med
Ano de publicação:
2009
Tipo de documento:
Article
País de afiliação:
Polônia