ABSTRACT
Case report Introduction: Good's syndrome (GS) represents an acquired adult-onset immunodeficiency associated with thymoma. GS affects patients over 40 yrs in form of recurrent infections especially with encapsulated bacteria, opportunistic viral and fungal invasions as a result of combined T/B cell deficiency. The imbalanced immunity may also provoke autoimmune phenomena and tumorigenesis. Case report: We present a 40-year- old male with a newly onset of dull thoracic pain and with no history of previous diseases. Chest CT revealed an anterior mediastinal mass in 2021, without lympadenopathy. A CT-guided core biopsy was suggestive for malignant thymoma, so the patient underwent total thymectomy. Histology indicated a thymoma of the AB type (WHO), and stage I. (Masaoka-Koga);(pT1a pNo). After surgery he was readmitted due to recurrent febrile respiratory tract infections, caused by Gram (-) bacteria or fungi;combination therapy of antibiotics and antifungal drugs was used. With suspicion of GS we determined immunoglobulin levels and the distribution of peripheral lymphocyte subsets. Hypogammaglobulinemia (IgG/A/M), and by flow cytometry markedly reduced peripheral B cells, and an inverse ratio of CD4+/CD8+ T cells were detected, confirming the diagnosis. Blast transformation assay indicated decreased T cell proliferation. Thus, following thymectomy, the patient exhibited severe T/B cell alterations with subsequent recurrent infections. Detailed autoantibody and complement analyses indicated no autoimmune laboratory abnormalities so far. There are still no effective protocols for GS therapy, except of antibiotic prophylaxis, preventive vaccination, and regular immunoglobulin replacement, so IVIG was introduced. As part of the follow-up repeated CT indicated no thymoma recurrence or metastasis. In December 2021 the vaccination refusing patient survived a severe bilateral organizing pneumonia secondary to SARS-CoV2. Conclusion(s): Incidence of the thymic epithelial tumor, thymoma is 0.15-0.33 cases/100.000/year. Depending on histology it could be linked to various immunological abnormalities. Appr. 0.2%-6% of thymomas corresponds to GS. GS, with a still elusive pathogenesis is considered as an uncommon combined immunodeficiency of adults with a variable phenotype and certain similarities to CVID. The prevalence is estimated appr. as 1/500.000. Combination of the high infection susceptibility and concomitant autoimmune diseases could make the diagnosis a challenging task.