# Clinical analysis of nine cases of hemophagocytic lymphohistiocytosis during pregnancy: characteristics, therapeutic strategies, and maternal-infant outcomes

## TL;DR

This study analyzes nine cases of hemophagocytic lymphohistiocytosis during pregnancy, focusing on clinical features, treatment strategies, and outcomes for mothers and infants.

## Contribution

The study provides a detailed clinical analysis of HLH during pregnancy, emphasizing treatment strategies and maternal-infant outcomes.

## Key findings

- Liver dysfunction was the most common initial symptom in two-thirds of patients.
- Termination of pregnancy was necessary for over half of the cases, with mixed fetal outcomes.
- High-dose corticosteroids were the primary treatment, with variable response rates and survival outcomes.

## Abstract

探讨妊娠期噬血细胞性淋巴组织细胞增生症（HLH）患者的临床特征和治疗结局。

回顾性分析2010年1月至2023年12月于江苏省人民医院就诊的9例妊娠期HLH患者的临床资料，总结其临床特点及治疗转归。

共纳入9例患者，妊娠早、中、晚期分别为1例（11.11％）、4例（44.44％）和4例（44.44％）；初次妊娠5例（55.56％），再次妊娠4例（44.44％）。6例（66.67％）患者以肝功能损伤为首诊表现。5例（55.56％）患者终止妊娠，其中2例（22.22％）妊娠晚期的患者胎儿存活、3例（33.33％）妊娠早、中期的患者胎儿死亡。7例（77.78％）接受糖皮质激素单药治疗，3例（33.33％）完全应答（CR）；1例（11.11％）患者采用环磷酰胺、长春新碱联合地塞米松方案化疗后CR；1例（11.11％）确诊后放弃治疗。中位随访5.0（0.6～103.7）个月，6例（66.67％）患者死亡，3例（33.33％）无病生存。

妊娠期HLH临床表现隐匿，肝功能异常为常见首发症状，早期诊断有助于改善患者预后。对于合并自身免疫性疾病及需要保障胎儿安全的妊娠期HLH患者，大剂量糖皮质激素可优先作为控制炎症风暴的治疗选择，终止妊娠时机需个体化决策。