Success with infliximab in treating refractory hemophagocytic lymphohistiocytosis
Success with infliximab in treating refractory hemophagocytic lymphohistiocytosis
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DOI：
10.1002/ajh.20462
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发表时间：
2006-01-01
影响因子：
12.8
通讯作者：
Harada, M
中科院分区：
文献类型：
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作者：
Henzan, T;Nagafuji, K;Harada, M
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见的疾病，其特征是发热、全血细胞减少、肝脾肿大、肝功能障碍和噬血细胞增多。一位29岁女性，于1996年被诊断为系统性红斑狼疮，于2002年6月初发展为HLH。在1.5个月的治疗期间，HLH仍然难治，包括皮质类固醇、环孢素、血浆置换、长春新碱和依托泊苷。然后给予英夫利西单抗（5 mg/kg/天）两次。第二次给药后，患者病情缓解。因为HLH本身不是肿瘤，而是一种不受控制的免疫反应，阻断参与反应的细胞因子应该具有治疗潜力。对于常规治疗无效的HLH患者，英夫利西单抗细胞因子治疗可能是一种有前途的选择。
Hemophagocytic lympholhistiocytosis (HLH) is a rare disorder characterized by fever, pancytopenia, hepatosplenomegaly, liver dysfunction, and hemophagocytosis. A 29-year-old woman, diagnosed with systemic lupus erythematosus in 1996, developed HLH in early June 2002. HLH remained refractory during 1.5 months of treatment including corticosteroid, cyclosporine, plasma exchange, vincristine, and etoposide. Infliximab (5 mg/kg/day) was then administered twice. After the second administration, the patient attained remission. Because HLH itself is not a neoplasm but an uncontrolled immune reaction, blocking cytokines involved in the reaction should have therapeutic potentials. For HLH patients not responding to conventional therapy, anticytokine treatment with infliximab may represent one of promising options.