Pure red cell aplasia with thymoma/T-cell large granular lymphocyte leukemia: two cases report and literatures review

Objective: To investigate the clinical and laboratory features of 2 cases of pure red cell aplasia (PRCA) with thymoma/T-cell large granular lymphocyte leukemia (T-LGLL), and to improve the recognition of the disease and the role of lymphocyte in its mechanism.

Methods: Two cases of PRCA with thymoma/T-LGLL were reported and the related literatures were reviewed.

Results: Case 1 was a 63-years old male with hemoglobin level of 54 g/L at admission. Case 2 was a 52-years old female with hemoglobin level of 79 g/L at admission. They were both diagnosed as PRCA with thymoma before admission to our hospital and had no benefit from their thymectomy. Further examinations in our hospital showed that CD3⁺CD4⁻CD8⁺CD57⁺ large granular lymphocytes amplified with clonal TCR rearrangement in their peripheral blood. The diagnosis of PRCA with thymoma/T-LGLL was clarified. Case 1 did not respond to any of the frontline therapies while case 2 responded completely to cyclosporine.

Conclusions: Both thymoma and T-LGLL could be the cause of secondary PRCA, lymphocyte proliferation may play critical role in the pathogenesis.