以肝损为首要表现的妊娠期噬血细胞综合征1例

Pregnancy induced hemophagocytic syndrome with liver damage as primary manifestation:A case report

噬血细胞综合征(HPS)是一类因免疫调节异常导致的过度炎症反应综合征。HPS病情进展迅速,预后凶险,而妊娠合并HPS导致病情更加复杂,为诊断及治疗均增加了难度,目前诊疗经验尚不成熟。本文报道了1例以皮疹、急性肝功能损害为首要表现的妊娠患者,剖宫产术后反复高热不退,行骨髓穿刺及PET-CT明确诊断噬血细胞综合征后,予激素及抗生素治疗,凶险病情逐步缓解,转危为安的案例。

Hemophagocytic syndrome(HPS)is a syndrome of excessive inflammatory response caused by abnormal immune regulation.HPS progresses rapidly and has dangerous prognosis.Pregnancy complicated with HPS makes the disease more complicated,which increases the difficulty of diagnosis and treatment,and the current diagnosis and treatment experience is still immature.This paper reports a case of a pregnant patient with acute liver function damage as the primary manifestation.After cesarean section,she suffered from recurrent high fever and was diagnosed with hemophagocytic syndrome by bone marrow puncture and PET-CT.After treatment with hormones and antibiotics,the dangerous condition was gradually relieved and the patient turned from danger to safety.