Hashim M S, Kordofani A Y, el Dabi M A
Department of Paediatrics and Child Health, Faculty of Medicine, University of Khartoum, Sudan.
Ann Trop Paediatr. 1997 Mar;17(1):61-5. doi: 10.1080/02724936.1997.11747865.
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.
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。