Fatal eosinophilic heart disease in a child with neurofibromatosis-1 complicated by acute lymphoblastic leukemia
Fatal eosinophilic heart disease in a child with neurofibromatosis-1 complicated by acute lymphoblastic leukemia
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DOI：
10.1007/s00380-004-0786-4
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发表时间：
2005-05-01
影响因子：
1.5
通讯作者：
Matsui, A
中科院分区：
文献类型：
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作者：
Horigome, H;Sumazaki, R;Matsui, A
我们报告一个小儿神经纤维瘤病-1（NF-1）并发急性淋巴细胞白血病和嗜酸性粒细胞增多症，导致多个终末器官损害。虽然临床症状，如发烧和咳嗽，注意到只有1周前入院，病情迅速恶化，抗白血病治疗前的致命结果。尸检显示广泛的肌内膜纤维化伴血栓性闭塞和冠状动脉再通。心肌组织未见白血病细胞浸润。当嗜酸性粒细胞增多症被诊断为NF-1患者，嗜酸性终末器官损害，特别是心脏受累，除了血液恶性肿瘤，应筛选，以便在疾病的早期阶段开始药物治疗。
We present a pediatric case of neurofibromatosis-1 (NF-1) complicated by acute lymphoblastic leukemia and hypereosinophilia, which caused multiple end-organ damage. Although clinical symptoms such as fever and coughing were noted only 1 week before admission, the condition deteriorated rapidly with a fatal outcome prior to antileukemic therapy. A postmortem examination demonstrated extensive endomyocardial fibrosis with thrombotic occlusion and recanalization of the coronary arteries. Leukemic cell infiltration was not seen in the cardiac tissue. When eosinophilia is diagnosed in patients with NF-1, eosinophilic end-organ damage, particularly cardiac involvement, in addition to hematological malignancies, should be screened for in order to start medical treatment at the early stage of the disease.