Wada Taizo, Nishiura Kashiku, Kuroda Mondo, Asai Erika, Vu Quang Van, Toma Tomoko, Niida Yo, Yachie Akihiro
Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan.
Brain Dev. 2012 May;34(5):376-9. doi: 10.1016/j.braindev.2011.07.005. Epub 2011 Jul 23.
We report on a 9-year-old boy who presented with acute encephalopathy and hemophagocytic lymphohistiocytosis (HLH). The patient was referred to our hospital because of fever, seizures, and decreased consciousness. He showed moderately elevated levels of proinflammatory cytokines in the cerebrospinal fluid and plasma, and clonal expansion of highly activated CD8⁺ T cells in the peripheral blood. These CD8⁺ T cells were found to be larger cells that stained positive for T-cell receptor Vβ13.6, and decreased shortly after steroid therapy. Our findings suggest that his acute encephalopathy was likely a clinical manifestation of HLH, and that immunophenotypic analysis may be helpful for early recognition of HLH in such rare encephalopathy.
我们报告了一名9岁男孩，他患有急性脑病和噬血细胞性淋巴组织细胞增生症（HLH）。该患者因发热、癫痫发作和意识减退被转诊至我院。他的脑脊液和血浆中促炎细胞因子水平中度升高，外周血中高度活化的CD8⁺ T细胞出现克隆性扩增。这些CD8⁺ T细胞为较大细胞，对T细胞受体Vβ13.6染色呈阳性，在类固醇治疗后不久数量减少。我们的研究结果表明，他的急性脑病可能是HLH的临床表现，免疫表型分析可能有助于在此类罕见脑病中早期识别HLH。
