Good syndrome associated with pure red cell aplasia in a patient with a history of thymoma: A case report and literature review

Abstract

Objectives

Good syndrome is a rare condition in which a thymoma is associated with hypogammaglobulinemia. It is also characterized by low or absent B-cells, CD4+ T-cell lymphopenia, inverted CD4/CD8+ T-cell relation, and repeated bacterial, viral, and fungal infections, as well as with various autoimmune disorders.

Case report

We report the case of a 62-year-old female patient who after resection of a thymoma developed constipation and recurrent lower respiratory and urinary tract infections. She underwent left hemicolectomy as well as symptomatic and antibiotic treatment. Two years later the patient develops refractory anemia; a bone marrow biopsy shows hypo-cellularity and no red cell precursors with negative CD 71 staining. The diagnosis of pure red cell precursor aplasia (PRCA) is made. Recurrent bacterial infections of the respiratory and urinary tracts continue, and therefore treatment with G and A immunoglobulins starts. Finally, the absence of B lymphocytes and inversion of CD4/CD8 relation confirms the diagnosis of Good syndrome associated with PRCA.

Discussion

The discovery of Good Syndrome and PRCA after the resection of a thymoma, as seen in the current case, is very unusual. This case illustrates both the rareness presentation of Good syndrome with PRCA and the persistent infections seen in these patients. Early recognition of these entities results in lower mortality and morbidity.