The Clinical Characteristics Of T Cell Large Granular Lymphocyte Leukemia Associated With Pure Red Cell Aplastic Anemia

Objective:To enhance the understanding of characteristics of acquired PRCA secondary to T-LGLL.Methods:Fourteen patients with T-LGLL associated with PRCA between 2000 and 2006 in our hospital were retrospectively analyzed.Results:Fourteen cases account for 34%(14/41) of T-LGLL and 42%(14/34) of PRCA. The median age at diagnosis was 61 years with equal gender distribution. PRCA associated with T-LGLL had indolent process, mainly presenting with anemia. Of those 14 patients,9 had mild to moderate splenomegaly, one heptomegaly and one lymphadenopathy. The median Hb level was 61.5g/L and the median WBC count was 4.3×109/L. The median percentage and count of LGL in peripheral blood was 35.5% and 1.9×109/L respectively. The median percentage of LGL in BM was 16.5%. Some patients had serologic abnormalities including positive RF and ANA. All the 12 cases who performed bone marrow cell cytogenetics examination showed were normal chromosome. With immunosuppressive therapy, the overall response was 91%. Conclusion:T-LGLL was the leading pathogenesis of acquired PRCA. PRCA in a setting of T-LGLL shares the similar clinical and laboratory feature to that of classical PRCA and has a good response to immunosuppressive therapy.