Fatal Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis with clonal karyotype abnormality.
Fatal Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis with clonal karyotype abnormality.
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致命性 Epstein-Barr 病毒相关的噬血细胞性淋巴组织细胞增多症伴克隆核型异常。
DOI：
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发表时间：
2000
影响因子：
2.1
通讯作者：
M. Yokoyama
中科院分区：
文献类型：
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作者：
E. Ito;J. Kitazawa;K. Arai;H. Otomo;Y. Endo;S. Imashuku;M. Yokoyama
我们报告一例eb病毒（EBV）相关的嗜血球淋巴组织细胞病（HLH）伴克隆核型异常。一名5岁男童因持续高热、肝肿大及全血细胞减少而入院。实验室数据显示凝血异常和严重的肝损害。通过Southern杂交检测到ebv感染细胞在骨髓中克隆性增殖，骨髓细胞出现克隆性染色体异常。患者虽按HLH94方案进行免疫化疗，但在诱导治疗期间疾病复发，患者死于弥散性血管内凝血病。考虑到这种侵袭性和致命性的临床过程，如果在治疗EBV-HLH患者时发现核型异常，采取强化治疗措施是很重要的。
We report a case of Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (HLH) with clonal karyotype abnormality. A 5-year-old boy was admitted to our hospital with persistent high-grade fever, hepatomegaly, and pancytopenia. Laboratory data disclosed a coagulation abnormality and severe liver damage. Clonal proliferation of EBV-infected cells was detected in the bone marrow by Southern hybridization, and bone marrow cells exhibited clonal chromosomal abnormality. Although the patient was treated with immunochemotherapy according to the HLH94 protocol, the disease recurred during the induction therapy, and the patient died of disseminated intravascular coagulopathy. Considering this aggressive and fatal clinical course, it is important to take intensive therapeutic measures if karyotype abnormality is noted in the treatment of EBV-HLH patients.