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目的探讨儿童噬血细胞综合征并多脏器功能不全的临床特征、治疗及预后。方法回顾性分析2008～2010年儿童重症监护室(PICU)收治的11例噬血细胞综合征并多脏器功能不全危重症患者的病因、临床特征、实验室检查以及治疗和转归。结果 (1)诱因:1例家族中既往有类似疾病,2例继发于细菌感染(大肠埃希菌和表皮葡萄球菌),4例继发于病毒感染(2例EB病毒,1例乙型肝炎病毒,1例巨细胞病毒),1例为非霍奇金T细胞淋巴瘤,1例为全身型幼年类风湿性关节炎,3例原因不明。(2)临床表现:反复发热伴肝脾肿大11例、呼吸窘迫7例、胸腹水8例、消化道出血7例;全血细胞减少10例、弥散性血管内凝血7例;低纤维蛋白原血症11例、肝功能异常11例、血清铁蛋白增高11例、高甘油三酯血症11例。(3)脏器损害累及肝脏、血液、呼吸、胃肠、心脏、肾脏、中枢等,以肝脏、凝血、呼吸最明显。(4)5例死于多脏器功能衰竭或原发病恶化,3例行血浆置换,6例获得缓解。结论 (1)噬血细胞综合征并多脏器功能不全病因复杂,病情凶险,进展快,受累脏器多,预后差,病死率高。(2)铁蛋白、甘油三酯及乳酸脱氢酶水平可反映重症噬血细胞综合征病情的严重程度。(3)血浆置换对噬血细胞综合征并严重多脏器功能不全患者是一种安全有效的辅助治疗方法 。
Objective To investigate the clinical features, treatment and prognosis of hemophagocytic syndrome in children with multiple organ dysfunction. Methods The etiology, clinical features, laboratory tests, treatment and prognosis of 11 patients with hemophagocytic syndrome and multiple organ dysfunction critically ill admitted to Children Intensive Care Unit (PICU) from 2008 to 2010 were retrospectively analyzed. Results (1) Incentives: One family had a previous history of similar diseases, two were secondary to bacterial infections (Escherichia coli and Staphylococcus epidermidis), four were secondary to viral infection (two with Epstein-Barr virus and one with type B Hepatitis virus, 1 case of cytomegalovirus), 1 case of non-Hodgkin’s T-cell lymphoma, 1 case of systemic juvenile rheumatoid arthritis, 3 cases of unknown cause. (2) clinical manifestations: recurrent fever with hepatosplenomegaly in 11 cases, respiratory distress in 7 cases, pleural effusion in 8 cases, 7 cases of gastrointestinal bleeding; pancytopenia in 10 cases, disseminated intravascular coagulation in 7 cases; low fibrinogen 11 cases of hemophilia, 11 cases of abnormal liver function, 11 cases of elevated serum ferritin and 11 cases of hypertriglyceridemia. (3) organ damage involving the liver, blood, respiratory, gastrointestinal, heart, kidney, central, etc., to the liver, clotting, breathing the most obvious. (4) 5 cases died of multiple organ failure or primary disease worsening, 3 cases of plasma exchange, 6 patients were relieved. Conclusions (1) hemophagocytic syndrome and multiple organ dysfunction complex, dangerous condition, rapid progress, more organs involved, poor prognosis, high mortality. (2) Ferritin, triglyceride and lactate dehydrogenase levels reflect the severity of severe hemophagocytic syndrome. (3) plasma exchange for hemophagocytic syndrome and severe multiple organ dysfunction is a safe and effective adjuvant therapy.