T细胞大颗粒淋巴细胞白血病合并纯红细胞再生障碍的临床特征

目的 提高对T细胞大颗粒淋巴细胞白血病(T-LGLL)合并纯红细胞再生障碍(PRCA)的认识.方法 回顾性分析我院2000年1月至2006年8月间确诊的T-LGLL合并PRCA患者临床及实验室检查特征.结果14例T-LGLL合并PRCA患者中男7例,女7例,中位年龄61岁.患者呈慢性病程,主要表现贫血症状,9例患者脾脏轻、中度肿大,1例患者同时伴轻度肝脏肿大,1例患者浅表淋巴结肿大.初诊时患者中位Hb 61.5 g/L,中位WBC 4.30(2.70～7.95)×109/L,中位淋巴细胞比例0.59(0.30～0.87),外周血大颗粒淋巴细胞中位比例和中位绝对值分别为0.36(0.14～0.77)和1.9(0.4～4.5)×109/L,骨髓有核细胞中大颗粒淋巴细胞中位比例0.165(0.085～0.410).部分患者血清学检查异常.12例患者常规染色体检查无异常.给予环孢素和(或)糖皮质激素为主的免疫抑制治疗,患者总有效率91%.结论 合并PRCA的T-LGLL患者临床及实验室特征与经典T-LGLL者相似,以贫血为突出表现,脾脏肿大多见.外周血白细胞正常或减少,大颗粒淋巴细胞相对增多.对免疫抑制治疗反应良好.

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 glucocoticold 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.