妊娠相关噬血细胞性淋巴组织细胞增生症临床诊疗分析  

Analysis of diagnosis and treatment of patients with hemophagocytic lymphohistiocytosis during pregnancy and postpartum

作　　者：王旖旎[1] 李硕[1] 黄文秋[1] 张嘉[1] 汤然[1] 张嘉[1] 吴林[1] 王昭 

机构地区：[1]首都医科大学附属北京友谊医院血液科,北京100050

出　　处：《临床和实验医学杂志》2015年第13期1057-1060,共4页Journal of Clinical and Experimental Medicine

基　　金：国家自然科学青年基金资助项目(编号:81401627);首都卫生发展科研专项青年基金资助项目(2编号:014-4-2025);北京市优秀人才项目D类

摘　　要：目的观察妊娠相关噬血细胞性淋巴组织细胞增生症(HLH)的临床特点,探索有效的治疗手段。方法对妊娠相关HLH患者的临床表现、实验室检查及疗效、预后进行综合分析。结果 8例妊娠相关HLH患者均为初次妊娠,2例患者发生于妊娠早期,6例患者发生于围产期;发热(100%)、脾大(75.0%)、消化系统症状(62.5%)是妊娠相关HLH的主要临床表现;所有患者均有s CD25的显著升高和NK细胞活性的下降,肝功能损害、高甘油三酯血症在妊娠相关HLH患者中的发生率更为突出;8例患者有2例患者病情进展迅速,于1月内死亡,6例获得长期生存并仍在随访中。结论妊娠期及产褥期存在异常的免疫调节可能是继发HLH的高危因素。及时正确的诊断和恰当的治疗有望改善预后。Objective To explore the features of hemophagocytic lymphohistiocytosis（ HLH） during pregnancy and postpartum and effective treatment. Methods A retrospective study was carried out to analyze the general information,clinical feature,laboratory findings,treatment and clinical outcomes of 8 patients with HLH during pregnancy and puerperium. Results Eight patients were all young primipara,75. 0% of them had the onset during perinatal period. The most common clinical features were fever（ 100%）,splenomegaly（ 75. 0%） and digestive symptoms（ 62. 5%）. High serum soluble CD25 and low NK cell activity were observed in all the patients,and hypertriglyceridemia and hepatic dysfunction were obvious. The total survival rate was 75%,two patients died during postpartum. Conclusion Immune disorder during pregnancy and puerperium may be one of the risk factors of secondary HLH. Correct early diagnosis and appropriate treatment may improve the prognosis of the patients.

关 键 词：妊娠期 产褥期 噬血细胞性淋巴组织细胞增生症 

分 类 号：R714.25[医药卫生—妇产科学]