摘要目的 探讨继发性噬血细胞性淋巴组织细胞增多症(sHLH)合并毛细血管渗漏综合征(CLS)的临床特征.方法 回顾性分析2015年1月至2017年12月江苏省人民医院收治的87例sHLH患者的临床及实验室资料,其中21例合并CLS(CLS-sHLH组),66例未合并CLS(非CLS-sHLH组).比较两组的临床表现、实验室检查、治疗及预后差异.结果 比较CLS-sHLH组和非CLS-sHLH组患者引起sHLH的病因,差异无统计学意义(P＞0.05).CLS-sHLH组患者ANC、血清纤维蛋白原、白蛋白较非CLS-sHLH组降低,而甘油三酯较非CLS-sHLH组升高(P值均＜0.05).CLS-sHLH组患者均有不同程度水肿、体重增加、低血压、低蛋白血症、少尿及多浆膜腔积液.其中15例患者CLS症状消失,CLS中位好转时间为7(5 ～14)d.6例患者CLS症状未消失,并于15(6～ 30)d死亡.CLS-sHLH组中位OS时间低于非CLS-sHLH组(75 d对未达到,P=0.031).结论 在sHLH患者中,CLS的发生可能与sHLH的原发病无明显相关.严重的ANC、纤维蛋白原及白蛋白减低,甘油三酯升高,可能是sHLH发生CLS的伴随症状.sHLH合并CLS时预后差.

abstractsObjective To investigate the clinical characteristics of secondary hemophagocytic lymphohistiocytosis (sHLH) complicated with capillary leak syndrome (CLS).Methods The clinical and laboratory data of 87 sHLH patients,who were treated in our hospital between January 2015 and December 2017,were retrospectively analyzed.Depending on whether they were complicated with CLS,21 sHLH patients were classified as the CLS-sHLH group,while 66 were classified as the non-CLS-sHLH group.The differences of clinical manifestations,laboratory tests,treatment and prognosis between the two groups were compared.Results There was no significant difference in the etiology of sHLH between the CLS-sHLH group and the non-CLS-sHLH group (P ＞ 0.05).The neutrophil,fibrinogen and albumin levels in the CLS-sHLH group were lower than those in the non-CLS-sHLH group,while the triacylglycerol levels were higher than those in the non-CLS-sHLH group (P＜ 0.05).Varying degrees of edema,weight gain,hypotension,hypoproteinemia,oliguria and multiple serous effusions were observed in the CLSsHLH group.Among them,there were 15 patients that CLS get improved,and the medial time of improvement was 7 (5-14) days.The other 6 patients did not get remission,while they died within 6-30 days.The median overall survival of the CLS-sHLH group was lower than that of the non-CLS-sHLH group (75 days vs not reached,P =0.031).Conclusions There may be no correlation between the cause of sHLH and the occurrence of CLS.Severity of neutropenia,fibrinogen and albumin levels,and triglyceride levels may be accompanied for sHLH patients complicated with CLS.Patients with sHLH who complicated with CLS have a poor prognosis.Active treatment of HLH and its primary disease,reasonable fluid replacement and oxygen supply are crucial,which can effectively control disease progression.