自身免疫病相关噬血细胞综合征22例临床分析

目的:通过对22例自身免疫病相关噬血细胞综合征(AAHS)患者临床资料分析,探讨本病的临床特点,提高对本病的认识。方法:对22例患者的临床表现、实验室检查、诊断、治疗方案及临床转归进行回顾性综合分析。结果:22例AAHS患者中,男4例,女18例,年龄16～62(33.73±10.63)岁,中位年龄31岁。基础病为成人Still病(AOSD)13例、系统性红斑狼疮(SLE)4例、干燥综合征(SS)1例、多发性肌炎(PM)1例、原发性胆汁性肝硬化(PBC)1例、组织细胞坏死性淋巴结炎(HNL)1例、皮肤血管炎1例。主要临床表现以持续高热(100%)、脾肿大(77%)最为多见。实验室检查所有患者均出现自然杀伤(NK)细胞活性下降、血清铁蛋白升高和清蛋白降低,其次较为常见的有噬血现象、转氨酶升高、可溶性CD25(sCD25)及乳酸脱氢酶水平升高,发生率均高于80%。外周血2系及以上血细胞减少占91%。22例AAHS患者经治疗后总体生存率为91%。结论:AAHS早期诊断和治疗预后较好。对于自身免疫病患者在常规方案治疗过程中,病情无明显缓解或出现急性加重时,同时伴有2系或以上血细胞减少、血清铁蛋白明显升高、肝功能损害严重等,应高度警惕噬血细胞综合征可能,积极完善相关检查明确诊断,及时积极有效治疗。

Clinical analysis of 23 patients with autoimmune disease associated hemophagocytic syndrome

Objective To analysis of the clinical data of 22 patients with autoimmune disease associated hemophagocytic syndrome （AAHS） for improving the awareness of the disease. Methods The clinical manifestation, laboratory examination, diagnosis, treatment and prognosis of 22 patients with AAHS were retrospectively analyzed. Results Of the 22 patients with AAHS, 4 were male and 18 were female, age ranging from 16-70 （35.30±12.85） years old. The underlying diseases were： 13 cases of adult onset Still＇s disease （AOSD）, 4 cases of systemic lupus erythematosus （SLE）, 1 case of Sjogren＇ s syndrome（SS）, 1 case of polymyositis （PM）, 1 case of primary biliary cirrhosis （PBC） , 1 case of histiocytic necrotizing lymphadenitis （HNL）,and 1 case of cutaneous vasculitis. The most common clinical features were persistent fever（100%） and splenomegaly （77%）. Laboratory examination revealed that all patients had decreased natural killer （NK） cell activity, elevated ferritin and decreased serum albumin, followed by the hemophagocytic phenomenon, elevations of aminotransferases, soluble CD25（sCD25） and lactate dehydrogenase（LDH）. Persistent fever（100%）, decrease of NK cell activity （100%）, elevation of ferritin （100%） and decrease of more than two lines of peripheral blood cells （91%） had high diagnostic sensitivity. The overall survival rate of the 22 patients was 91%. Conclusions With early diagnosis and treatment, the prognosis of AAHS is quite good. If the patient with autoimmune disease has no significant remission or exacerbation during the course of conventional treatment, and is accompanied by decrease of more than two lines of blood cells, elevation of serum ferritin and severe impairment of liver function, the presence of AAHS should be considered and the diagnosis be confirmed by relevant examinations and given effective treatment.