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[Clinical analysis of Good's syndrome in 10 cases].

OBJECTIVE
To summarize the clinical characteristics of Good's syndrome so as to standardize its diagnostic and therapeutic strategy.


METHODS
Ten cases of Good's syndrome treated at our hospital from January 2000 to January 2011 were analyzed retrospectively. Their clinical manifestations, accessory examinations, treatment and prognosis were evaluated.


RESULTS
There were 3 males and 7 females with an average age of (49 ± 11) years old. Their major symptoms were pulmonary infection (n = 6), diarrhea (n = 4), involvement of central nervous system (CNS) (n = 4) and polyarticular pain (n = 3). Hypogammaglobulinaemia and abnormal subsets of lymphocytes were the most common laboratory changes. Besides imaging studies revealed that all of them were diagnosed as thymoma. No significant efficacy was observed after thymoma resection. Clinical improvements were demonstrated after the treatment with regular intravenously administered immunoglobulin (IVIG) in 4 of them. Eight patients were followed up. Four of them died from pulmonary infection and involvement of CNS.


CONCLUSION
As a rare disease in adults, Good's syndrome is characterized by thymoma associated with immunodeficiency and a lack of specific clinical manifestations. IVIG at regular intervals is the most effective way of improving the clinical symptoms and reducing the patient mortality. However, the prognosis remains poor since most patients fail to receive a regular therapy of IVIG.