Parrott Jessica, Shilling Alexander, Male Heather J, Holland Marium, Clark-Ganheart Cecily A
Division of Maternal Fetal Medicine, Department of Obstetrics and Gynecology, University of Kansas Medical Center, 3901 Rainbow Boulevard, Kansas City, KS 66160, USA.
Division of Hematologic Malignancies and Cellular Therapeutics, Department of Internal Medicine, University of Kansas Medical Center, 3901 Rainbow Boulevard, Kansas City, KS 66160, USA.
Case Rep Obstet Gynecol. 2019 Feb 12;2019:9695367. doi: 10.1155/2019/9695367. eCollection 2019.
Hemophagocytic lymphohistiocytosis (HLH) is a rare disease that can be fatal in pregnancy. We report two cases of severe HLH that highlight etoposide use in pregnancy.
CASE 1: 28-year-old G2P1 with lupus presented at 18 weeks with acute hypoxic respiratory failure, hepatic dysfunction, leukopenia, thrombocytopenia, and elevated ferritin. Bone marrow biopsy confirmed HLH. Etoposide and corticosteroid treatment was initiated per HLH protocol; however clinical status declined rapidly. Fetal demise occurred at 21 weeks and she subsequently suffered a massive cerebral vascular accident. She was transitioned to comfort measures and the patient deceased.
CASE 2: 37-year-old G4P3 presented at 25 weeks with fever, acute liver failure, thrombocytopenia, and elevated ferritin. HLH treatment was initiated, including etoposide, and diagnosis confirmed with liver biopsy. Fetal growth restriction was diagnosed at 27 weeks. Delivery occurred at 37 weeks. The neonate was found to be CMV positive despite negative maternal serology.
The addition of etoposide to corticosteroid use is a key component in HLH treatment of nonpregnant individuals. While this is usually avoided in pregnancy, the benefit to the mother may outweigh the potential harm to the fetus in severe cases and it should be strongly considered.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见疾病，在孕期可能致命。我们报告两例严重HLH病例，突出了依托泊苷在孕期的使用情况。
病例1：一名28岁、孕2产1的狼疮患者在孕18周时出现急性低氧性呼吸衰竭、肝功能障碍、白细胞减少、血小板减少及铁蛋白升高。骨髓活检确诊为HLH。按照HLH治疗方案开始使用依托泊苷和皮质类固醇治疗；然而临床状况迅速恶化。孕21周时胎儿死亡，随后她发生了大规模脑血管意外。她被改为采取舒适护理措施，患者最终死亡。
病例2：一名37岁、孕4产3的患者在孕25周时出现发热、急性肝衰竭、血小板减少及铁蛋白升高。开始进行HLH治疗，包括使用依托泊苷，肝脏活检确诊。孕27周时诊断出胎儿生长受限。孕37周时分娩。尽管母亲血清学检查为阴性，但新生儿被发现巨细胞病毒呈阳性。
在非孕期HLH治疗中，在使用皮质类固醇的基础上加用依托泊苷是关键组成部分。虽然孕期通常避免使用，但在严重情况下，对母亲的益处可能超过对胎儿的潜在危害，应予以充分考虑。
