What is Good’s syndrome? Immunological abnormalities in patients with thymoma
- 1Department of Immunology, St Mary’s Hospital, Praed Street, London W2 1NY, UK
- 2Department of Immunology, Oxford Radcliffe NHS Trust, John Radcliffe Hospital, Oxford OX3 9DV, UK
- Dr P Kelleher, Department of Immunology, St Mary’s Hospital, Praed Street, London W2 1NY, UK;
- Accepted 5 August 2002
- CMV, cytomegalovirus
- cpm, counts per minute
- CT, computed tomography
- CVID, common variable immune deficiency
- HIV, human immunodeficiency virus
- IL, interleukin
- PHA, phytohaemagglutinin
- XLA, X linked agammaglobulinaemia
Good’s syndrome (thymoma with immunodeficiency) is a rare cause of combined B and T cell immunodeficiency in adults. The clinical characteristics of Good’s syndrome are increased susceptibility to bacterial infections with encapsulated organisms and opportunistic viral and fungal infections. The most consistent immunological abnormalities are hypogammaglobulinaemia and reduced or absent B cells. This disorder should be treated by resection of the thymoma and immunoglobulin replacement to maintain adequate trough IgG values.
The association between the presence of a thymoma and immunodeficiency was first recognised in 1954 by Dr Robert Good, who described a case of thymoma and hypogammaglobulinaemia in an adult.1 Although there are no formal diagnostic criteria for this disorder it is classified as a distinct entity by the expert committee of the World Health Organisation/International Union of Immunological Societies on primary immunodeficiencies.2 Good’s syndrome was noted in 7% of adults with primary antibody deficiency attending a chest clinic,3 although this figure is probably influenced by referral bias, and the incidence of this condition in patients with primary antibody deficiency who are on immunoglobulin replacement treatment is more likely to be 1–2%. In patients with thymoma the incidence of hypogammaglobulinaemia is 6–11%.4,5 The cause and pathogenesis of this disorder are unknown, although there is some evidence that the basic defect may be in the bone marrow (pre-B cell arrest, impaired maturation of erythroid and myeloid precursors in some patients).
Patients with Good’s syndrome usually present in the 4th or 5th decade of life. In a literature review of the infectious complications of this disorder the mean age of initial symptoms was 56 years (range, 29–75) and the mean age for the recognition of thymoma and hypogammaglobulinaemia was 62 years (range, 41–79).6 The interval between the start of initial symptoms and the diagnosis of …