Introduction: Good’s syndrome (GS) is a rare disease (1.5 cases per million) presenting with thymoma and hypogammaglobulinemia. Although it has been suggested that it might be a subset of Common Variable Immunodeficiency, the knowledge of the disease is hampered by the incomplete and inconsistent observations reported. A more detailed dissection of the immune cells in a significant set of patients would contribute to understand the pathophysiology of the disease.
Methods: Up to 350 immune subpopulations were analyzed in six GS patients (53-79 years) from 4 different hospitals in parallel to 49 age-matched controls using next-generation flow cytometry.
Results: All patients consistently presented with lack of B-cells (0.05). Interestingly, counts of naïve CD8+ and TCRγδ+ T-cells were normal, meanwhile central and transitional memory CD8+ and TCRγδ+ T-cells were reduced (p≤0.01). However, total TCRγδ+ T-cells tends to be higher (p=0.07) due to statistically significant expanded terminally differentiated cells (p≤0.01). All the other immune subsets analyzed were within the normal range.
Conclusions: Together with the lack of B-cells, a complex profile of alterations was observed in GS patients including a large spectrum of defects in the adaptative (CD4+ T-cells) and innate cells (NK-cells, eosinophils, neutrophils, basophils, DCs). In contrast, expanded TCRγδ+ T-cells and normal counts of Th1 CD4+ and CD8+ T-cells were observed.