妊娠期噬血细胞性淋巴组织细胞增生症进展为暴发性肝炎。
Hemophagocytic lymphohistiocytosis in pregnancy evolving towards fulminant hepatitis.
作者信息
Simard Camille, Tardif Marie-Lou
机构信息
Department of Medicine, McGill University, Montreal, Canada.
Division of Obstetric Medicine, Department of Obstetrics and Gynecology, CHU Sainte-Justine, Montreal, Canada.
出版信息
Obstet Med. 2022 Sep;15(3):208-211. doi: 10.1177/1753495X21999914. Epub 2021 Mar 24.
BACKGROUND
Hemophagocytic lymphohistiocytosis is a rare disorder, especially in pregnancy, characterized by excessive immune activation leading to hemophagocytic activity.
CASE REPORT
A 34-year-old woman presented at 31 weeks' gestation with fever, cytopenias, hyperferritinemia and fulminant hepatitis. Bone marrow biopsy was non-contributory, and no trigger was identified. Liver biopsy confirmed the diagnosis of hemophagocytic lymphohistiocytosis, and she received dexamethasone and etoposide, which was changed to anakinra to allow breastfeeding. Prompt diagnosis and treatment of hemophagocytic lymphohistiocytosis is crucial to the survival of pregnant women, but no consensus exists regarding the most appropriate therapy during pregnancy.
CONCLUSION
Hemophagocytic lymphohistiocytosis is life-threatening and associated with significant morbidity and mortality. Important treatment considerations in pregnancy include maternal health, fetal prematurity and treatment toxicity. Anakinra has been successfully used in pregnancy and provides a promising alternative.
背景
噬血细胞性淋巴组织细胞增生症是一种罕见疾病，在孕期尤其罕见，其特征为过度免疫激活导致噬血细胞活性。
病例报告
一名34岁女性在妊娠31周时出现发热、血细胞减少、高铁蛋白血症和暴发性肝炎。骨髓活检无诊断价值，未发现触发因素。肝活检确诊为噬血细胞性淋巴组织细胞增生症，她接受了地塞米松和依托泊苷治疗，后改为阿那白滞素以允许母乳喂养。及时诊断和治疗噬血细胞性淋巴组织细胞增生症对孕妇的生存至关重要，但对于孕期最合适的治疗方法尚无共识。
结论
噬血细胞性淋巴组织细胞增生症危及生命，与显著的发病率和死亡率相关。孕期重要的治疗考虑因素包括母体健康、胎儿早产和治疗毒性。阿那白滞素已成功用于孕期，提供了一种有前景的替代方案。
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