Machowicz Rafał, Janka Gritta, Wiktor-Jedrzejczak Wieslaw
Department of Hematology, Oncology and Internal Diseases, Medical University of Warsaw, ul. Banacha 1a, 02-097 Warsaw, Poland.
Department of Paediatric Haematology and Oncology, University Medical Centre Hamburg Eppendorf, Martinistrasse 52, 20246 Hamburg, Germany.
Crit Rev Oncol Hematol. 2017 Jun;114:1-12. doi: 10.1016/j.critrevonc.2017.03.023. Epub 2017 Mar 23.
Differential diagnosis of hemophagocytic lymphohistiocytosis (HLH; hemophagocytic syndrome) and sepsis is critically important because the life-saving aggressive immunosuppressive treatment, required in the effective HLH therapy, is absent in sepsis guidelines. Moreover, HLH may be complicated by sepsis. Hyperinflammation, present in both states, gives an overlapping clinical picture including fever and performance status deterioration. The aim of this review is to provide aid in this challenging diagnostic process. Analysis of clinical features and laboratory results in multiple groups of patients (both adult and pediatric) with either HLH or sepsis allows to propose criteria differentiating these two conditions. The diagnosis of HLH is supported by hyperferritinemia, splenomegaly, marked cytopenias, hypofibrinogenemia, low CRP, characteristic cytokine profile and, only in adults, hypertriglyceridemia. In the presence of these parameters (especially the most characteristic hyperferritinemia), the other HLH criteria should be assessed. Genetic analyses can reveal familial HLH. Hemophagocytosis is neither specific nor sensitive for HLH.
噬血细胞性淋巴组织细胞增生症（HLH；噬血细胞综合征）与脓毒症的鉴别诊断至关重要，因为有效的HLH治疗所需的挽救生命的积极免疫抑制治疗并不包含在脓毒症治疗指南中。此外，HLH可能并发脓毒症。两种状态下均存在的过度炎症会呈现出包括发热和身体状况恶化在内的重叠临床表现。本综述的目的是为这一具有挑战性的诊断过程提供帮助。对多组患有HLH或脓毒症的患者（包括成人和儿童）的临床特征和实验室结果进行分析，有助于提出区分这两种疾病的标准。HLH的诊断依据包括高铁蛋白血症、脾肿大、明显血细胞减少、低纤维蛋白原血症、低CRP、特征性细胞因子谱，且仅在成人中存在高甘油三酯血症。在出现这些参数（尤其是最具特征性的高铁蛋白血症）时，应评估其他HLH标准。基因分析可揭示家族性HLH。噬血细胞现象对HLH既不具有特异性也不具有敏感性。
