OBJECTIVE: 

Hemophagocytic lymphohistiocytosis is a hyperinflammatory syndrome that often requires critical care support and remains difficult to diagnose. These guidelines are meant to aid in the early recognition, diagnosis, supportive care, and treatment of patients with hemophagocytic lymphohistiocytosis in ICUs.

DATA SOURCES: 

The literature searches were performed with PubMed (MEDLINE).

STUDY SELECTION: 

Keywords and medical subject headings terms for literature search included “macrophage activation syndrome,” hemophagocytic lymphohistiocytosis,” and “hemophagocytic syndrome.”

DATA EXTRACTION: 

The Histiocyte Society developed these consensus recommendations on the basis of published reports and expert opinions with level of evidence provided for each recommendation. They were endorsed by the Society of Critical Care Medicine.

DATA SYNTHESIS: 

Testing for hemophagocytic lymphohistiocytosis should be initiated promptly in all patients admitted to ICUs with an unexplained or disproportionate inflammatory response, especially those with rapid clinical deterioration. Meeting five or more of eight hemophagocytic lymphohistiocytosis 2004 diagnostic criteria serves as a valuable diagnostic tool for hemophagocytic lymphohistiocytosis. Early aggressive critical care interventions are often required to manage the multisystem organ failure associated with hemophagocytic lymphohistiocytosis. Thorough investigation of the underlying triggers of hemophagocytic lymphohistiocytosis, including infections, malignancies, and autoimmune/autoinflammatory diseases, is essential. Early steroid treatment is indicated for patients with familial hemophagocytic lymphohistiocytosis and is often valuable in patients with acquired hemophagocytic lymphohistiocytosis (i.e., secondary hemophagocytic lymphohistiocytosis) without previous therapy, including macrophage activation syndrome (hemophagocytic lymphohistiocytosis secondary to autoimmune/autoinflammatory disease) without persistent or relapsing disease. Steroid treatment should not be delayed, particularly if organ dysfunction is present. In patients with macrophage activation syndrome, whose disease does not sufficiently respond, interleukin-1 inhibition and/or cyclosporine A is recommended. In familial hemophagocytic lymphohistiocytosis and severe, persistent, or relapsing secondary macrophage activation syndrome, the addition of prompt individualized, age-adjusted etoposide treatment is recommended.

CONCLUSIONS: 

Further studies are needed to determine optimal treatment for patients with hemophagocytic lymphohistiocytosis in ICUs, including the use of novel and adjunct therapies.

中文翻译：

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危重儿童和成人噬血细胞性淋巴组织细胞增多症的识别、诊断和治疗的共识指南

客观的： 

噬血细胞性淋巴组织细胞增多症是一种高炎症综合征，通常需要重症监护支持并且仍然难以诊断。这些指南旨在帮助 ICU 中噬血细胞性淋巴组织细胞增多症患者的早期识别、诊断、支持护理和治疗。

数据源： 

文献检索是通过 PubMed (MEDLINE) 进行的。

研究选择： 

文献检索的关键词和医学主题词包括“巨噬细胞活化综合征”、“噬血细胞性淋巴组织细胞增多症”和“噬血细胞综合征”。

数据提取： 

组织细胞协会根据已发表的报告和专家意见制定了这些共识建议，并为每项建议提供了证据水平。他们得到了重症监护医学协会的认可。

数据综合： 

对于所有因不明原因或不成比例的炎症反应而入住 ICU 的患者，尤其是临床快速恶化的患者，应立即开始噬血细胞性淋巴组织细胞增多症检测。满足 2004 年 8 项噬血细胞性淋巴组织细胞增多症诊断标准中的 5 项或以上可作为噬血细胞性淋巴组织细胞增多症的有价值的诊断工具。通常需要早期积极的重症监护干预来治疗与噬血细胞性淋巴组织细胞增多症相关的多系统器官衰竭。彻底调查噬血细胞性淋巴组织细胞增多症的潜在诱因，包括感染、恶性肿瘤和自身免疫/自身炎症性疾病，至关重要。早期类固醇治疗适用于患有家族性噬血细胞性淋巴组织细胞增多症的患者，对于患有获得性噬血细胞性淋巴组织细胞增多症（即继发性噬血细胞性淋巴组织细胞增多症）而未接受过治疗的患者通常很有价值，包括巨噬细胞活化综合征（继发于自身免疫/自身炎症性疾病的噬血细胞性淋巴组织细胞增多症），但没有持续性或复发性疾病。不应延迟类固醇治疗，特别是在存在器官功能障碍的情况下。对于巨噬细胞激活综合征患者，其疾病没有充分缓解，建议使用白细胞介素-1 抑制剂和/或环孢素 A。对于家族性噬血细胞性淋巴组织细胞增多症和严重、持续性或复发性继发性巨噬细胞活化综合征，建议添加及时个体化、年龄调整的依托泊苷治疗。

结论： 

需要进一步的研究来确定 ICU 中噬血细胞性淋巴组织细胞增多症患者的最佳治疗方法，包括使用新型疗法和辅助疗法。