Pure red cell aplasia associated with thymoma: a report of a single-center experience

Pure red cell aplasia (PRCA) associated with thymoma is relatively rare, and relevant reports are limited. We investigated the clinical features and outcomes of PRCA associated with thymoma in this study.

A retrospective review of all PRCA patients who underwent surgical resection of thymoma from April 1, 2004, to December 31, 2015, was performed.

We experienced eight patients with PRCA among 146 patients who underwent surgical resection of thymoma. Extended thymectomy (n=4) and thymectomy (n=4) were performed for thymoma. Regarding the WHO classification of thymoma, the subtypes were type B2 or B3 in seven patients, and the stage of thymoma was advanced in seven patients. Complete resection was achieved macroscopically in only five patients. Recurrence of thymoma occurred in four patients who underwent complete resection. PRCA was diagnosed after surgical resection of thymoma in six patients (range 1-101 months, median 56.5 months). Cyclosporine was used for PRCA in six patients. Pneumonia of treatment-related complications due to cyclosporine occurred in all patients. The follow-up period ranged from 13-147 months (median 54.5 months) after the PRCA diagnosis. Three patients obtained complete remission of anemia by cyclosporine. Although one patient was able to stop taking cyclosporine because of complete remission of anemia, transfusion was needed due to relapse of PRCA. Five patients died, with the main causes of death diagnosed as pneumonia (n=4) and cardiac failure (n=1).

PRCA associated with thymoma was diagnosed postoperatively in three-quarter of patients. We should be alert for the occurrence of PRCA even after resection of thymoma, especially in patients with incomplete resection or advanced disease. Cyclosporine was effective for PRCA, but treatment-related complications occurred, particularly pneumonia. As treatment for PRCA associated with thymoma and its complications were combined in a complex manner, treating PRCA associated with thymoma can be quite difficult.