Background and Aims

The Good Syndrome (GS) refers to a combination of thymoma and adult-onset immunodeficiency that consists of low or absent B cells and hypogammaglobulinemia in peripheral blood. The pathogenesis of the immunodeficiency in GS remains elusive. A bone marrow defect is suggested by B and T cell lymphopenia, and the frequent coexistence of eosinopenia. Pure red cell aplasia or neutropenia occasionally develops.

Methods

Presentation of 3 GS cases with its clinical features, diagnosis, therapeutic approach and evolution.

Results

Case 1: Female, 43 years-old, diagnosed in 2018 with invasive thymoma with pleural metastasis that later developed oral paraneoplastic pemphigus and myasthenia gravis. The analysis revealed - B lymphopenia and panhypogammaglobulinemia, all associated to recurrent respiratory and urinary infections, anemia that required blood transfusion, severe neutropenia and thrombocytopenia.

During the next year she required several hospitalizations because of respiratory infections and gastrointestinal symptoms, with poor evolution developing sepsis with exitus despite IVIG therapy.

Case 2: Female, 61 years-old, diagnosed with thymoma, with complete resection in 2014. Anemia, severe neutropenia and panhypogammaglobulinemia treated with IVIG. Hospitalized with interstitial lymphoid pneumonitis in 2015 treated with Corticosteroids and Rituximab with a good clinical response. Stable evolution.

Case 3: Male, 69 years-old, diagnosed with GS agammaglobulinemy associated to thymoma (resected) treated with IVIG, associated to chronic diarrhea (Campylobacter yeyuni) and ankylosing spondylitis. Thrombocytopenia and leucopenia with progressive anemia. Also diagnosed with idiopathic cirrhosis Child A with portal hypertension. Stable evolution.

Conclusions

A diversity of the clinical presentation is observed in the GS that requires a multidisciplinary management of these patients.