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儿童结核病与骨髓纤维化:一份报告

Tuberculosis and myelofibrosis in children: a report.

作者信息

Hashim M S, Kordofani A Y, el Dabi M A

机构信息

Department of Paediatrics and Child Health, Faculty of Medicine, University of Khartoum, Sudan.

出版信息

Ann Trop Paediatr. 1997 Mar;17(1):61-5. doi: 10.1080/02724936.1997.11747865.

DOI:10.1080/02724936.1997.11747865
PMID:9176580
Abstract

Seven children, six boys and a girl, aged from 2 to 15 years with proven myelofibrosis are reported. The clinical presentation in each of them was more or less similar with weight loss, moderate or low-grade fever, and abdominal distension with pain or discomfort for some months. They had hepatosplenomegaly. The spleens, enlarged to more than 6 cm below the costal margin, were smooth, firm and not tender. There was a variable degree of generalized lymphadenopathy. They were diagnosed as myelofibrosis associated with tuberculosis. The clinical response to anti-tuberculous chemotherapy was remarkable. Extensive search should be made for evidence of tuberculosis in children presenting with myelofibrosis.

摘要

报告了7名年龄在2至15岁之间经证实患有骨髓纤维化的儿童,其中6名男孩和1名女孩。他们每个人的临床表现大致相似,有体重减轻、中度或低热,以及腹胀伴疼痛或不适数月。他们有肝脾肿大。脾脏肿大至肋缘下超过6厘米,表面光滑、质地坚硬且无压痛。有不同程度的全身淋巴结肿大。他们被诊断为与结核病相关的骨髓纤维化。抗结核化疗的临床反应显著。对于出现骨髓纤维化的儿童,应广泛寻找结核病的证据。

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Congenital macrothrombocytopenia with focal myelofibrosis due to mutations in human G6b-B is rescued in humanized mice.由于人类 G6b-B 突变导致的先天性巨血小板减少症伴局灶性骨髓纤维化在人源化小鼠中得到挽救。
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