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以发热为首发症状的儿童原发性髓系肉瘤1例报道并文献复习
袁远宏,张慧,肖政辉,罗海燕,杨龙贵,段尉,范江花
0
(湖南省儿童医院,湖南长沙 410007)
摘要:
髓系肉瘤(myeloid sarcoma,MS)是由髓系原始细胞或未成熟髓系细胞在髓外增生和浸润所形成的肿块,常伴随急性髓系白血病(AML)出现,也可出现于骨髓增殖性疾病(MPD)或骨髓增生异常综合征(MDS) 患者病程中,罕见于AML发生之前。外周血和骨髓均无白血病表现,称为原发性MS。MS 可出现在不同组织或器官中,可发生在任何年龄,但多见于青年和儿童,男性多于女性。MS临床表现无特异性,诊断比较困难,误诊率较高。笔者现报告l例以发热为首发症状的原发性MS 患儿,探讨其临床及实验室检查特点,并复习相关文献,以提高临床对MS的认识
关键词:  髓系肉瘤  白血病  发热  首发症状
DOI:doi:10.13407/j.cnki.jpp.1672-108X.2018.11.001
基金项目:十二五国家科技支持项目,编号2012BAI04B01;第二批湖南省卫生厅重大疾病研究中心组建项目,湘卫科教发也2013页6号。
Primary Myeloid Sarcoma in Children with Fever as the First Symptom: A Case Report and Literature Review
Yuan Yuanhong, Zhang Hui, Xiao Zhenghui, Luo Haiyan, Yang Longgui, Duan Wei, Fan Jianghua
(Hunan Children's Hospital, Hunan Changsha 410007, China)
Abstract:
Myeloid sarcoma (MS) is a mass formed by extramedullary proliferation and infiltration of myeloid primordial cells or immature myeloid cells, often accompanied by acute myeloid leukemia (AML), which may also occur in the course of patients with myeloproliferative disease (MPD) or myelodysplastic syndrome (MDS), rarely before AML occurs. There is no leukemia in peripheral blood and bone marrow, which is called primary MS. MS can occur in different tissues or organs and at any age, but is more common in the youth and children, men more than women. The clinical manifestations of MS are non-specific, the diagnosis is difficult and the rate of misdiagnosis is relatively high. The authors reported a case of primary MS with fever as the first symptom, to explore its clinical and laboratory characteristics and to review the relevant literature to improve the understanding of the disease.
Key words:  myeloid sarcoma  leukemia  fever  starting symptoms

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