Variable clinical characteristics and laboratory results in five patients with Chinese Good's syndrome (thymoma and hypogammaglobulinemia): an 8-year retrospective analysis in a university hospital in China

Hypogammaglobulinemia Thymectomy Common Variable Immunodeficiency
DOI: 10.1186/s12865-021-00441-9 Publication Date: 2021-08-03T05:02:37Z
ABSTRACT
Abstract Background Good's syndrome (GS) is a rare secondary immunodeficiency disease presenting as thymoma and hypogammaglobulinemia. Due to its rarity, the diagnosis of GS often missed. Methods We used hospital information system retrospectively screen hypogammaglobulinemia patients at First Affiliated Hospital Wenzhou Medical University from Apr 2012 2020. The clinical, laboratory, treatment, outcome data for these were collected analyzed. Results Among 181 screened patients, 5 with identified; 3 had confirmed diagnoses GS, other 2 did not have recorded in system. A retrospective review clinical characteristics, laboratory results, follow-up undiagnosed GS. All presented pneumonia, recurrent skin abscesses, cough expectoration, 1 patient oral lichen planus diarrhea, tuberculosis granulomatous epididymitis. In years after mild symptoms, all received irregular intravenous immunoglobulin (IVIG) treatment. As course progressed, symptoms worsened, but partly resolved IVIG patients. However, 4 died due comorbidities. Conclusion should be investigated possible who present hypogammaglobulinemia, especially those opportunistic infections, chronic or planus.
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