慢性淋巴细胞白血病并发自身免疫性血细胞减少临床分析

Clinical analysis of autoimmune cytopenia in chronic lymphocytic leukaemia

目的：探讨慢性淋巴细胞白血病（CLL）并发自身免疫性血细胞减少（AIC）的临床特征、生物学特征、治疗及转归。方法：回顾性分析我院诊断治疗的CLL并发AIC患者的临床特征、生物学特征、治疗方法及转归,并复习相关文献。结果：56例CLL患者中6例（11%）并发AIC,其中5例并发自身免疫性溶血性贫血（AIHA）,1例并发纯红细胞再生障碍性贫血。4例在经过化疗后并发AIC,中位时间为7（2~40）个月;2例在初诊时并发AIHA。中位年龄为58.5岁,5例为男性;4例为C期,2例为B期。中位外周血淋巴细胞计数为29.25G/L。经过激素、静脉滴注丙种球蛋白、利妥昔单抗或联合化疗后5例达缓解,其中3例随着原发病CLL的复发又再次并发AIC。1例患者经激素和化疗后Coombs＇试验转阴,因CLL进展骨髓衰竭并发鲍曼不动杆菌感染而死亡。随访至2012年7月,2例死亡,2例失访,2例存活。结论：CLL并发AIC与男性、高龄、进展期及高的淋巴细胞计数相关。激素为CLL并发AIC的一线治疗,对于CLL进展期的患者联合化疗（如利妥昔单抗＋氟达拉滨＋环磷酰胺）效果更好。CLL并发AIC的复发率较高,控制原发病可降低AIC的复发率。

Objective：To investigate the clinical and biological characteristics,treatment and prognosis of auto- immune cytopenia（AIC）in patients with chronic lymphocytic leukemia（CLL）.Method：The clinical and biological characteristics,treatment and prognosis of patients with CLL and AIC were retrospectively analyzed.Furthermore, the relevant literatures were reviewed.Result：Six of 56CLL patients（11%）had AIC,of whom five patients had autoimmune hemolytic anemia and one had pure red cell aplasia.Four patients developed AIC after therapy with a median time of seven months（range from 2to 40months）since treatment exposure and two patients were detected AIHA at diagnosis.The median age was 58.5years and five patients were male.There were four cases at Binet stage C and two cases at Binet stage B.The median peripheral blood lymphocyte count was 29.25G/L.Five pa- tients achieved a response after the treatment of corticosteroids,intravenous immunoglobulin,rituximab with or without chemotherapy.However,three patients relapsed with the recurrence of CLL.One patient＇s Coombs＇ test be- came negative after therapy with corticosteroids and chemotherapy,but he did not escape death for the progression of original disease and the Acinetobacter baumannii infection.Until followed up to July 2012,two cases died,two cases lost of follow-up and two cases were alive.Conclusion：CLL patients with AIC are associated with male,older age,a high lymphocyte count and advanced clinical stage.Corticosteroid is the first-line treatment for patients with CLL and AIC,and combination chemotherapy（rituximab＋fludarabine＋cyclophosphamide）has better curative effects for advanced phase patients.CLL patients with AIC may show higher relapse rate,actively controlling the primary disease will decrease the recurrent rate of AIC.