Erduran E, Gedik Y, Sen Y, Yildiran A
Department of Pediatrics, Karadeniz Technical University Faculty of Medicine, Trabzon, Turkey.
Turk J Pediatr. 2000 Apr-Jun;42(2):168-70.
Infection-associated hemophagocytic syndrome (IAHS) is a form of the reactive hemophagocytic syndrome. IAHS is associated with viral, bacterial, fungal, mycobacterial, rickettsial and protozoal infections and with various malignant neoplasms. A more accurate designation for this acquired form of the syndrome is reactive hemophagocytic syndrome (HS). Reactive HS is characterized by malaise, fever, hepatosplenomegaly, lymphadenopathy, cytopenia, hypertriglyceridemia, hypofibrinogenemia and hemophagocytosis. Cyclosporin A, VP-16, high-dose steroids, and intravenous immunoglobulin (IVIG) have been used in the treatment of IAHS. Here, a 10-year-old girl with reactive HS due to possible viral infection was treated successfully with cyclosporin A and IVIG. Fever disappeared on the third day, complete blood count reached normal levels on the sixth day and hepatosplenomegaly disappeared on the ninth day after treatment. We believe cyclosporin A and IVIG may be used in the treatment of reactive HS, at least in selected patients. Further studies are required to confirm its role as first-line therapy for children with IAHS.
感染相关性噬血细胞综合征（IAHS）是反应性噬血细胞综合征的一种形式。IAHS与病毒、细菌、真菌、分枝杆菌、立克次体及原生动物感染以及各种恶性肿瘤有关。这种获得性综合征更准确的命名是反应性噬血细胞综合征（HS）。反应性HS的特征为不适、发热、肝脾肿大、淋巴结病、血细胞减少、高甘油三酯血症、低纤维蛋白原血症及噬血细胞现象。环孢素A、依托泊苷、大剂量类固醇及静脉注射免疫球蛋白（IVIG）已用于IAHS的治疗。在此，一名因可能的病毒感染导致反应性HS的10岁女孩经环孢素A和IVIG治疗成功。治疗后第三天发热消失，第六天全血细胞计数恢复正常水平，第九天肝脾肿大消失。我们认为环孢素A和IVIG可用于反应性HS的治疗，至少在部分患者中如此。需要进一步研究以证实其作为儿童IAHS一线治疗方法的作用。
