[Precise diagnosis and treatment of hemophagocytic lymphohistiocytosis guided by cytokine patterns].

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening entity which presents as multi-organ dysfunction caused by overactivated T cell and macrophage and overwhelming hyperinflammation. Cytokine storm is the key pathophysiology of HLH. Due to the diversity of the underlying diseases, the difference of disease severity and the non-specific clinical manifestation, the early diagnosis and standardized treatment is a great challenge for clinicians. According to previous clinical studies, serum cytokine measurement is helpful for rapid diagnosis of HLH and the differentiation of HLH from other diseases caused by hypercytokinemia, and it perfoms well to clarify subtypes of HLH, to stratify patients with different risks and to predict the prognosis and outcome. Thus, serum cytokine measurement could be a useful tool for the precise diagnosis and treatment of HLH.噬血细胞综合征（HPS）或噬血细胞性淋巴组织细胞增生症（HLH）是一组由各种原因导致T淋巴细胞和单核巨噬细胞过度增殖活化引起全身高炎症反应的临床综合征。细胞因子风暴是该病的关键环节。由于病因复杂、疾病严重程度差异大、临床表现非特异性等原因，该病的早期诊断和规范化治疗较为困难。细胞因子在HLH的早期快速诊断、与其他细胞因子风暴相关疾病的鉴别、亚型判别、分层治疗、疗效和预后判断等方面均具有重要意义。本文就HLH相关细胞因子谱类型及其在与各类发热性疾病的鉴别诊断、HLH的分层治疗和治疗过程监测中的应用等方面进行阐述，以进一步提高HLH的精准诊治水平。.