复旦学报（医学版）FudanUnivJMedSci2023Mar.，50（2）妊娠合并噬血细胞综合征3例报道时冬冬程琰张庆英△（复旦大学附属妇产科医院产科上海200082）【摘要】噬血细胞综合征（hemophagocyticsyndrome，HPS）是一类因免疫调节异常导致的过度炎症反应综合征。妊娠合并HPS发病率低，且缺乏特异性临床表现，容易误诊而延误治疗。本文回顾性分析了复旦大学附属妇产科医院收治的3例妊娠合并HPS的孕妇临床资料：其中2例患者首发症状均为妊娠中晚期出现的皮肤瘙痒及肝功能受损，而终止妊娠后开始出现反复高热，肝脾肿大，肝酶升高，两系或三系血细胞减少，血清铁蛋白显著升高，骨髓穿刺提示HPS；另一例患者首发症状为孕中期淋巴结肿大及发热，在抗生素治疗后仍反复高热，三系血细胞减少，肝酶升高，骨髓穿刺提示HPS。3例病例经大剂量糖皮质激素治疗后均获得缓解，预后较好。妊娠合并HPS病情复杂，容易误诊，出现持续高热、抗生素治疗无效同时伴有肝酶升高、两系或三系血细胞减少、肝脾肿大等相关临床表现时，应考虑到妊娠合并HPS的可能，及时诊断及合理治疗可改善预后。【关键词】妊娠；噬血细胞综合征（HPS）；临床表现；诊断【中图分类号】R714.25【文献标志码】Bdoi：10.3969/j.issn.1672-8467.2023.02.025Pregnancycomplicatedwithhemophagocyticsyndrome：3casesreportSHIDong-dong，CHENGYan，ZHANGQing-ying△（DepartmentofObstetrics，ObstetricsandGynecologyHospital，FudanUniversity，Shanghai200082，China）【Abstract】Hemophagocyticsyndrome（HPS）isasyndromeofexcessiveinflammatoryresponsecausedbyabnormalimmuneregulation.However，theincidenceofHPSinpregnancyislow，andthelackofspecificclinicalmanifestationsmakesiteasytomisdiagnoseanddelaytreatment.Thisstudyretrospectivelyanalyzedtheclinicaldataof3pregnantwomenwithHPSwhowereadmittedtoObstetricsandGynecologyHospital，FudanUniversity.Theprimarysymptomsofcase1andcase2werepruritusandimpairedliverfunctioninlatetrimesterofpregnancy，butafterterminationofpregnancy，thepatientsdevelopedrepeatedhighfever，hepatosplenomegalyandelevationofliverenzymes，threeortwolinesofbloodcellsdecreased，serumferritinincreasedsignificantly，bonemarrowpuncturesuggestedHPS.Theprimarysymptomsofcase3werelymphadenopathyandfeverduringthesecondtrimester...
