期刊
AMERICAN JOURNAL OF HEMATOLOGY
卷 81, 期 1, 页码 59-61出版社
WILEY
DOI: 10.1002/ajh.20462
关键词
hemophagocytic lymphohistiocytosis; tumor necrosis factor-alpha; infliximab; cytokines
类别
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.
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