疑难病杂志2018，Vol.17Issue(7)：725-728,4.DOI:10.3969/j.issn.1671-6450.2018.07.018
妊娠相关噬血细胞综合征6例临床分析
Clinical analysis of 6 cases of pregnancy related hemophagocytic syndrome
摘要
Abstract
Objective To observe the clinical characteristics of pregnancy related hemophagocytic syndrome (HPS) and improve the level of understanding and diagnosis of HPS. Methods A retrospective analysis of the causes, clinical mani-festations, laboratory tests, treatment schemes and clinical outcomes of 6 patients with HPS was performed. Results Of the 6 patients with pregnancy related HPS, 3 were again pregnant, and the other 3 were the first pregnancy. 1 case occurred in the early pregnancy, and 2 cases occurred in the puerperium. All 6 cases were mainly caused by infection, 2 cases with virus in-fection, 2 cases with bacterial infection, 1 case with adult Still, and 1 case with unknown cause. The main clinical manifesta-tion was persistent high fever (100% ). All patients had significantly elevated sCD25 and decreased NK cell activity. Besides blood cell depletion,liver dysfunction,hypertriglyceridemia,and elevated ferritin were associated with a high incidence in preg-nancy related HPS patients. All 6 patients received long term survival and were still followed up. Conclusion There is an ab-normal immune regulation during pregnancy and puerperium. It is easy to induce inflammatory response and secondary HPS. For patients with persistent fever, abnormal liver function, and hemocyte reduction in pregnancy and puerperium, HPS should be taken into consideration, and the timely and correct diagnosis and treatment can obtain good prognosis.关键词
妊娠期/产褥期/噬血细胞综合征/临床表现/治疗方案Key words
Pregnancy/Puerperium/Hemophagecytic syndrome/Clinical characteristics/Therapeutic plan引用本文复制引用
任美欣,单晶,韩莹,边新渠,黄春洋,杜晓菲,张小丹,刘燕敏,黄云丽,廖慧钰..妊娠相关噬血细胞综合征6例临床分析[J].疑难病杂志,2018,17(7):725-728,4.基金项目
北京市丰台区卫生计生系统科研项目(2017-63) （2017-63）
