挑战：巨噬细胞活化综合征和脓毒症相关噬血细胞淋巴组织细胞增生症

Pediatric macrophage activation syndrome/sepsis-associated hemophagocytic lymphohistiocytosis：a diagnostic and therapeutic challenge

无论原发性还是继发性噬血细胞淋巴组织细胞增生症都可能是致死性的，早期发现和及时治疗是改变结局的关键。虽然在基因缺陷和免疫发病机制研究进展的基础上形成的免疫化疗和定向免疫治疗使部分患儿的生存率得到了改善，但在ICU降低噬血细胞淋巴组织细胞增生症的病死率依然面临挑战。持续发热、伴随凝血异常的特殊性肝功能不良、高甘油三脂血症、血细胞减少和异常升高的血清铁蛋白是重要的诊断线索。明确患者自然杀伤细胞在脓毒症和自身免疫性疾病不同阶段的情况将引导创新免疫介入治疗的实现。

It is an important turning point that early diagnosis and prompts initiation of adequate treatment improve the outcomes of primary or secondary hemophagocytic lymphohistiocytosis （HLH） which is a rare life-threatening syndrome. Even though the survival rate of the patients with HLH has been improved because of the immunochemotherapy or targeted immunotherapy based on new findings in the immunopathogenesis and the genetic defects ,reducing the mortality will be main challenge for the pediatricians in pediatric intensive care unit. All of the clinical features are significant clues for the diagnosis, including continuing fever, specific liver dysfunction with coagulation abnormalities, pancytopenia, hypertriglyceridemia and hyperferritinemia. Defining patients＇ natural killer ceils status during the different stages of sepsis and autoimmune diseases will be guiding innovative immunointerventions.