Bottari Gabriella, Murciano Manuel, Merli Pietro, Bracaglia Claudia, Guzzo Isabella, Stoppa Francesca, Pardeo Manuela, Nunziata Joseph, Del Bufalo Francesca, Genuini Leonardo, De Benedetti Fabrizio, Locatelli Franco, Cecchetti Corrado
Division of Rheumatology, Department of Pediatrics, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Pediatric Emergency Department, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Blood Purif. 2022;51(5):417-424. doi: 10.1159/000517471. Epub 2021 Aug 3.
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition characterized by a state of hyperinflammation. Blood purification techniques can blunt the inflammatory process with a rapidly relevant nonselective effect on the cytokine storm, thus potentially translating into survival benefit for these patients. In this cohort, we evaluated the impact of hemoadsorption with CytoSorb combined with continuous kidney replacement therapy used as adjunctive therapy in 6 critically ill children with multiple organ dysfunction due to HLH. In our series, we found a reduction in inflammatory biomarkers in patients with HLH secondary to infection. Ferritin, one of the most important bedside biomarkers of HLH, showed a reduction in most of the treated patients. The same results were found measuring interleukin-6 and interleukin-10. The same patients showed hemodynamic stabilization measured by the Vasopressor-Inotropic-Score, and reduction in the organ disease score measured with the Pediatric Logistic Organ Dysfunction score. In our cohort, mortality was less than expected based on the Pediatric Index of Mortality 3 score at pediatric intensive care unit admission. Our study shows that hemoperfusion could be a valuable therapeutic option in HLH: stronger scientific evidence is needed to confirm our preliminary experience.
噬血细胞性淋巴组织细胞增生症(HLH)是一种以过度炎症状态为特征的危及生命的病症。血液净化技术可以通过对细胞因子风暴产生迅速相关的非选择性作用来抑制炎症过程,从而有可能为这些患者带来生存益处。在本队列研究中,我们评估了使用CytoSorb进行血液吸附联合连续性肾脏替代疗法作为辅助治疗对6例因HLH导致多器官功能障碍的危重症儿童的影响。在我们的系列研究中,我们发现继发于感染的HLH患者的炎症生物标志物有所减少。铁蛋白是HLH最重要的床边生物标志物之一,在大多数接受治疗的患者中均有所降低。在检测白细胞介素-6和白细胞介素-10时也发现了相同的结果。同样这些患者通过血管活性药物-正性肌力药物评分显示血流动力学稳定,并且通过小儿逻辑器官功能障碍评分测得的器官疾病评分降低。在我们的队列中,根据小儿重症监护病房入院时的小儿死亡率指数3评分,死亡率低于预期。我们的研究表明血液灌流可能是HLH中一种有价值的治疗选择:需要更强有力的科学证据来证实我们的初步经验。