Haytoglu Zeliha, Yazici Nalan, Erbay Ayse
*Department of Pediatrics, Cukurova University Faculty of Medicine †Department of Pediatrics, Division of Pediatric Oncology, Adana Teaching and Medical Research Center, Baskent University Faculty of Medicine, Adana, Turkey.
J Pediatr Hematol Oncol. 2017 Mar;39(2):e106-e109. doi: 10.1097/MPH.0000000000000740.
Because of the acute and life-threatening course of the hemophagocytic lymphohistiocytosis (HLH) syndrome, International Histiocyte Society guidelines recommend chemoimmune therapy for the treatment of both primary and secondary HLH (sHLH). To manage children with sHLH, instead of HLH-2004 protocol we considered less immunosuppressive/cytotoxic approach. We assessed 12 children who fulfilled the diagnostic criteria for sHLH between January 2009 and March 2015. Multivariate Cox regression analysis showed that ferritin levels (hazard ratio=1.02, P=0.006), pediatric logistic organ dysfunction scores (hazard ratio=1.01, P=0.001) were the predictors of the survival. The hospital survival was 83% for patients with sHLH who were treated with less immunosuppressive therapy. In conclusion initiation of HLH-specific therapy for the patients with hyperferritinemia-associated sHLH should be delayed while awaiting resolution of systemic inflammation with less immunosuppressive therapy.
由于噬血细胞性淋巴组织细胞增生症（HLH）综合征病程急且危及生命，国际组织细胞协会指南推荐采用化学免疫疗法治疗原发性和继发性HLH（sHLH）。为了治疗sHLH患儿，我们考虑采用免疫抑制/细胞毒性较小的方法，而非HLH-2004方案。我们评估了2009年1月至2015年3月期间符合sHLH诊断标准的12名儿童。多变量Cox回归分析显示，铁蛋白水平（风险比=1.02，P=0.006）、儿科逻辑器官功能障碍评分（风险比=1.01，P=0.001）是生存的预测因素。接受免疫抑制治疗较少的sHLH患者的医院生存率为83%。总之，对于与高铁蛋白血症相关的sHLH患者，在等待通过免疫抑制作用较小的疗法解决全身炎症之前，应推迟启动HLH特异性治疗。
