Fujiwara F, Hibi S, Imashuku S
Department of Pediatrics, Children's Research Hospital, Kyoto Prefectural University of Medicine, Japan.
Am J Pediatr Hematol Oncol. 1993 Feb;15(1):92-8. doi: 10.1097/00043426-199302000-00012.
The study was performed to clarify in the hemophagocytic syndrome (HPS) how cytokinemia plays a role in its pathogenesis and if cytokinemia is of prognostic value.
Serum concentrations of ferritin, interferon (IFN)-gamma, soluble interleukin-2 (IL-2) receptor, IL-6, and other cytokines were determined during the acute phase of the HPS in 29 children and three adults. Data comparing malignancy-associated (MAHS; n = 17) and infection-associated hemophagocytic syndrome (IAHS; n = 15) and those comparing surviving and fatal cases were assessed.
Hyperferritinemia and hypercytokinemia were present in all patients with HPS. Eleven of the 17 MAHS and three of the 15 IAHS cases were fatal (p < 0.05). No significant difference in cytokine concentrations was observed between MAHS and IAHS.
In terms of cytokine effect on patient outcome, serum concentrations of IL-6 > 300 ng/L and IFN-gamma concentrations > 30 U/ml or soluble IL-2 receptor (sIL-2R) concentrations > 10,000 U/ml were considered to reflect a poor prognosis in HPS patients.
本研究旨在阐明在噬血细胞综合征（HPS）中细胞因子血症如何在其发病机制中发挥作用以及细胞因子血症是否具有预后价值。
测定了29名儿童和3名成人HPS急性期血清中铁蛋白、干扰素（IFN）-γ、可溶性白细胞介素-2（IL-2）受体、IL-6及其他细胞因子的浓度。评估了比较恶性肿瘤相关性（MAHS；n = 17）和感染相关性噬血细胞综合征（IAHS；n = 15）的数据以及比较存活和死亡病例的数据。
所有HPS患者均存在高铁蛋白血症和高细胞因子血症。17例MAHS患者中有11例死亡，15例IAHS患者中有3例死亡（p < 0.05）。MAHS和IAHS之间未观察到细胞因子浓度的显著差异。
就细胞因子对患者预后的影响而言，血清IL-6浓度> 300 ng/L、IFN-γ浓度> 30 U/ml或可溶性IL-2受体（sIL-2R）浓度> 10,000 U/ml被认为反映了HPS患者的预后不良。
