Pure red cell aplasia with thymoma/T-cell large granular lymphocyte leukemia: two cases report and literatures review / 中华血液学杂志

ABSTRACT

<p><b>OBJECTIVE</b>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.</p><p><b>METHODS</b>Two cases of PRCA with thymoma/T-LGLL were reported and the related literatures were reviewed.</p><p><b>RESULTS</b>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.</p><p><b>CONCLUSION</b>Both thymoma and T-LGLL could be the cause of secondary PRCA, lymphocyte proliferation may play critical role in the pathogenesis.</p>