小剂量IL-2对获得性噬血细胞综合征的治疗

Low dose of IL-2 for the treatment of acquired hemophagocytic syndrome

对获得性噬血细胞综合征(HPS)患者持续应用100万单位白介素(IL)-2,每天1次,皮下注射,联合抗感染、激素等常规治疗;观察患者在治疗前后临床症状和体征的变化。患者在持续应用小剂量IL-2治疗后,高热等异常临床表现逐渐消失,4周后血象及各项免疫相关检查基本恢复正常,病情一度稳定,但停药后又出现反复,进行性加重,6个月后死于"恶性组织细胞病"引起的多脏器功能衰竭。利用IL-2特有的双向免疫调节活性,可能为治疗HPS,尤其是感染相关的获得性HPS提供一个新的方式。

To explore the effect of low dose IL-2 on the immunologic function in the acquired hemophagocytic syn- drome （HPS） patient and the therapeutic value. One case of acquired HPS patient who had been initially diagnosed received continuously IL-2 at 100 million unit subcutaneously once a day and anti-infection, prednisone convention- al treatment before and after. The clinical symptoms and laboratory parameters were significantly improved after four weeks but became deteriorated after withdrawal and the patient died of multiple system organ failure after six months. This investigation showed the two-ways regulative effects of IL-2 may provide a new treatment method in HPS patients, especially in acquired HPS associated with infection.