Henzan Tomoko, Nagafuji Koji, Tsukamoto Hiroshi, Miyamoto Toshihiro, Gondo Hisashi, Imashuku Shinsaku, Harada Mine
Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, Fukuoka, Japan.
Am J Hematol. 2006 Jan;81(1):59-61. doi: 10.1002/ajh.20462.
Hemophagocytic lymphohistiocytosis (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.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见的疾病，其特征为发热、全血细胞减少、肝脾肿大、肝功能障碍和噬血细胞现象。一名29岁女性，于1996年被诊断为系统性红斑狼疮，在2002年6月初患上HLH。在包括皮质类固醇、环孢素、血浆置换、长春新碱和依托泊苷在内的1.5个月治疗期间，HLH仍然难治。随后给予英夫利昔单抗（5毫克/千克/天）两次。第二次给药后，患者病情缓解。由于HLH本身不是肿瘤，而是一种失控的免疫反应，阻断参与该反应的细胞因子应该具有治疗潜力。对于对传统疗法无反应的HLH患者，用英夫利昔单抗进行抗细胞因子治疗可能是一种有前景的选择。
