OKT4 epitope deficiency in a patient with thymoma and hypogammaglobulinemia

Abstract

A 61-year-old male stonecutter was in excellent health until December 1988 when he was admitted to Miyagikenritsu Semine Hospital with an abnormality on chest X-ray film. Chest X-ray film revealed a left anterior mass that was confirmed on chest CT scan and MRI. Biochemical examinations and serum protein electrophoresis demonstrated hypoproteinemia (5.4 g/dl) and hypogammaglobulinemia (7.9%, 0.43 g/dl). Lymphocyte subset analysis showed OKT4 epitope deficiency (OKT4 0.1% and Leu3a 28.4%), and decrease of CD4/CD8 ratio (0.65). An encapsulated anterior mediastinal mass and the thymus were resected on January 9, 1989, without difficulty. Histology revealed a thymoma with a spindle cell epithelial component. OKT4 epitope deficiency in a patient with Good's syndrome (thymoma with hypogammaglobulinemia) was our diagnosis. There was no change in the patient's serum immunoglobulin level after thymectomy. Fifteen cases of Good's syndrome have been reported in Japan, but this is the first report of OKT4 epitope deficiency with Good's syndrome in Japan.