The Immunological considerations in patients with Thymoma : A case report with lessons beyond resection

Abstract

Abstract Background Thymic epithelial tumours (TET) are a rare group of immunologically active thymic malignancies associated with paraneoplastic and extremely rare autoimmune conditions. Good Syndrome (GS) is one such rare adult-onset immunodeficiency associated with Thymoma. The incidence is 0.15 cases per 100 000 population years, immunophenotype is variable and it is associated with significant morbidity and poor ten-year survival estimated at only 30%. Case presentation A 22-year-old female with recurrent infections, ocular limited Myasthenia Gravis and large thymoma underwent elective total thymectomy via median sternotomy. Surgical resection was unremarkable however post operatively she developed a chylothorax with pleural fluid cultures positive for Candida Albicans requiring prolonged antibiotic therapy. Her post operative course was further complicated by shingles, myopericarditis, brachioradialis myositis, and bulbar myasthenic crises. Immunological work up showed pan-hypogammaglobulinemia IgG 3.8 (7–16 g/L), IgA < 0.05 (0.7-4 g/L), IgM 0.12 (0.4–2.3 g/L), absent pneumococcal humoral response, reduced T cells (CD3 + 0.43x109/L with a normal CD4/8 ratio) but normal total B cells (CD19+/20 + 0.11 x109/L). She was diagnosed with Good Syndrome and is actively managed with long term Intravenous Immunoglobulin therapy (IVIG). Conclusions The immunological sequelae of TET, GS in particular, are poorly understood and under recognized in the surgical community. This case report highlights diagnostic complexities and emphasizes a need early, comprehensive, multidisciplinary perioperative management.