[The clinical characteristics of T cell large granular lymphocyte leukemia associated with pure red cell aplasia].

OBJECTIVE

To analyze the characteristics of acquired pure red cell aplasia (PRCA) secondary to T cell large granular lymphocyte leukemia (T-LGLL).

METHODS

Fourteen patients with T-LGLL associated with PRCA between 2000 and 2006 in our hospital were retrospectively analyzed.

RESULTS

The median age at diagnosis was 61 years with equal gender distribution. The PRCA had indolent process, mainly presenting with anemia. Of the 14 patients, 9 had mild to moderate splenomegaly, one hepatomegaly and one lymphadenopathy. The median Hb level was 61.5 g/L and the median WBC count 4.3 x 10(9)/L. The median percentage and count of LGL in peripheral blood were 0.36 and 1.9 x 10(9)/L respectively. The median percentage of LGL in BM was 0.165 (0.085 - 0.410). Some patients had serologic abnormalities. All the 12 cases with available bone marrow cell cytogenetics showed normal karyotypes. With cyclosporine A or glucocorticoid immunosuppressive therapy, the overall response was 91%.

CONCLUSION

T-LGLL was one of the major causes of acquired PRCA. This type of PRCA has the similar clinical and laboratory feature to that of other type of PRCA and has a good response to immunosuppressive therapy.