难治性噬血细胞综合征并发多脏器功能衰竭一例ICU抢救体会

噬血细胞综合征（HPS），又名噬血细胞性淋巴组织细胞增多症（HLH），是由活化的淋巴细胞和组织细胞增殖失控并分泌大量炎性细胞因子而引起的过度炎症反应，危及生命。本文介绍1例难治性噬血细胞综合征并发多脏器功能衰竭患者的抢救过程，通过对病史和治疗经过的回顾，抢救时机与细节的分析，挽救性治疗这一理念的实践等方面，探讨重症难治性HPS在重症监护病房（ICU）抢救中的策略与体会。本例患者抢救成功的关键点不仅仅是抓住了初治的早期以及挽救治疗的及时性、有效性，更是与ICU的实时监护和及时有效处理有关。ICU抢救中有效的感染控制和全面的脏器支持可明显改善HPS的阶段性治疗结局，为患者的存活提供可能。

A case of refractory hemophagocytic syndrome combined with multiple organ failure - ICU rescue experience

Hemophagocytic syndrome (HPS), also known as hemophagocytic lymphohistiocytosis (HLH), is a lifethreatening condition of severe hyperinflammation caused by the uncontrolled proliferation of activated lymphocytes andhistiocytic secreting high amounts of inflammatory cytokines. This article describes a case of refractory hemophagocytic syndrome combined with multiple organ failure. Through the review of medical history and treatment process, analysis of rescue occasion and details, and the practice on the concept of salvage therapy, our purpose is to discuss the strategy and experience that we have gained from rescuing severe refractory hemophagocytic syndrome in ICU. The key to the success rescue of this case is that we not only have carried out timely and effective early stage of initial treatment and salvage treatment, but also have had real-time monitoring and timely and effective treatment in ICU. Based on the effective infectioncontrol and comprehensive organ support, the phased treatment results of patients with HPS can be significantly improved,which provides possibility for HPS patients’survival.