000372 - A Case Report of Late-Onset Immunodeficiency: Good's Syndrome

Aim: Good's syndrome (GS) is a rare disease involving thymoma and hypogammaglobulinemia, typically seen in adults aged 40-70, and is a cause of late-onset primary immunodeficiency (PID). Recurrent pulmonary and gastrointestinal infections are common in GS. Moreover, bacterial, fungal, and viral opportunistic infections occur frequently due to defects in humoral and cellular immunity. Beyond infections, GS patients may present autoimmune disorders such as myasthenia gravis, oral lichen planus, and pure red cell aplasia. Here, we report a GS case diagnosed with hypogammaglobulinemia at an older age, presenting with candida esophagitis and chronic diarrhea.

Case: A 78-year-old female with chronic obstructive pulmonary disease, thymoma, and coronary artery disease experienced nausea, vomiting, and diarrhea for 7 years, losing 20 kg in 3 months. Mouth sores and fungal infections have appeared in the last 6 months. Gastroenterology evaluation revealed candida esophagitis via endoscopy, with normal ileocolonoscopic findings. To investigate the etiology of chronic diarrhea, the patient admitted to the infectious diseases department and presented isospora belli cysts in the stool. The patient was consulted by us and examined for immunodeficiency. Three years ago, a nodular lesion measuring 24x41 mm was detected in the right upper lobe on the thoracic CT (Figure 1) scan upon the detection of right hilar fullness in the P.A. chest X-ray. The result of the transthoracic needle biopsy was consistent with thymoma. The patient was recommended for surgery but refused it. No parental consanguinity or family history of chronic illnesses was noted. One brother had coronary artery disease, and the siblings had no history of immune deficiency. The examinations conducted by us revealed hypogammaglobulinemia in the patient (Table 1).  Good's syndrome was considered in the patient and intravenous immunglobulin treatment was started. After treatment, the diarrhea condition subsided.

Conclusion: Good's syndrome should be considered in every patient with thymoma and a history of frequent infections. Detailed immunological evaluation should be performed in these patients. Early diagnosis and treatment are important given that the prognosis of GS is worse compared to CVID patients. Increased awareness may enhance early recognition of this syndrome and prevent mortality.