儿童恶性肿瘤相关性噬血细胞综合征24例临床分析

Clinical features and prognosis of malignancy-associated hemophagocytic lymphohistiocytosis in children:a clinical analysis of 24 cases

目的探讨儿童恶性肿瘤相关性噬血细胞综合征(MAHS)的临床特征和预后。方法针对24例MAHS的原发病、临床特点与转归进行回顾性分析。结果 24例患儿中11例(46%)为肿瘤诱发,13例(54%)为化疗相关。原发病为急性白血病的有17例、淋巴瘤6例、神经母细胞瘤1例。临床表现以发热、呼吸系统症状、肝脾肿大为前三位常见症状。血细胞减少、血清铁蛋白升高、乳酸脱氢酶水平升高为前三位常见的实验室异常。22例给予HLH-2004方案治疗、2例放弃治疗。24例中死亡18例、失访1例、存活5例,生存时间3 d至2年4个月不等,中位生存期28 d。结论儿童恶性肿瘤相关性噬血细胞综合征临床表现多样,治疗效果极差。

Objective To investigate the clinical features and prognosis of malignancy-associated hemophagocytic lymphohistiocytosis（MAHS） in children. Methods A retrospective analysis was performed for the primary diseases, clinical features, and prognosis of 24 children with MAHS. Results Among the 24 children, 11（46%） had MAHS induced by tumor and 13（54%） had chemotherapy-associated MAHS. As for primary diseases, 17 children had acute leukemia, 6 had lymphoma, and 1 had neuroblastoma. The most common clinical manifestations were pyrexia, respiratory symptoms, and hepatosplenomegaly. The most common laboratory abnormalities were hemocytopenia, elevated serum ferritin, and elevated lactate dehydrogenase. Of the 24 children, 22 were treated according to the HLH-2004 protocol and 2 gave up treatment; 18 children died, 1 was lost to follow-up, and 5 survived. The survival time ranged from 3 days to 2 years and 4 months（median 28 days）. Conclusions Children with MAHS have various clinical features and extremely poor treatment outcomes.