Immunological disturbances in Good’s syndrome

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.

Immunoglobulin Replacement Therapy in Secondary Hypogammaglobulinemia

“…Often, hypogammaglobulinemia persists after the treatment of the underlying disease. There are some reports on the efficacy of IgRT in these patients ( 57 , 58 ).…”

Regulation of thymic T regulatory cell differentiation by TECs in health and disease

“…It is widely believed that the thymus gland plays a very limited role in the maintenance of immune function or self-tolerance mechanisms in adults, but autoimmune complications are common in thymoma disease and/or immunodeficiency patients. 116 The most common autoimmune disease associated with thymoma is myasthenia gravis.…”

A 73-year-old woman with persistent diarrhea and onychomycosis

“…Laboratory studies were obtained, and the results are recorded in Tables 1 and 2 [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] and absolute B-cell count was Ͻ1 cells/mm 3 (reference range, 15-495 cells/mm 3 ). The absolute T-cell count was 159 cells/mm 3 (reference range, 603-2990 cells/mm 3 ), absolute T-helper cell count was 58 cells/mm 3 (reference range, 45-1500 cells/mm 3 ), absolute T cytotoxic cell count 99 cells/ mm 3 (reference range, 120 -895 cells/mm 3 ), and CD4/ CD8 ratio was 0.59 (reference range, 1-3).…”