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儿童获得性再生障碍性贫血:100例患者的回顾

Acquired aplastic anemia in children: a review of 100 patients.

作者信息

Chuansumrit A, Hathirat P, Isarangkura P

机构信息

Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.

出版信息

Southeast Asian J Trop Med Public Health. 1990 Jun;21(2):313-20.

PMID:2237600
Abstract

A retrospective study of acquired aplastic anemia in 100 Thai children treated with testosterone and prednisolone during 1969 to 1987 is reported. The age ranged from 3-14.5 years (mean 10.3 years). The male to female ratio was 2.3:1. The duration of follow up ranged from 1-17 year (mean 5 years). Prior exposure to possible etiologic agents was found in 36% : antipyretics, Ya-chood, insecticides, benzene, chloramphenicol and paint. The presenting symptoms were bleeding and anemia 72%, fever with either bleeding or anemia 28%. The common sites of bleeding were purpura, epistaxis, gum and teeth, and the gastrointestinal tract. All patients received appropriate supportive treatment and testosterone combined with prednisolone. The results of treatment were evaluated in 80 cases who were followed up until death or at least one year after the diagnosis. Two-thirds of the patients presented with laboratory findings similar to severe aplastic anemia; the fatality rate was 50% (40/80). Most of them succumbed to infection or bleeding in the first 6 months after diagnosis. The recovery rate was 50% with complete, partial and initial responses in 28%, 17% and 5% respectively. The late malignancy rate was 2%. For severe aplastic anemia or for patients who have poor prognostic factors, early approaches of bone marrow transplantation or antilymphocyte globulin administration should be considered.

摘要

本文报道了一项对1969年至1987年间接受睾酮和泼尼松龙治疗的100名泰国儿童获得性再生障碍性贫血的回顾性研究。年龄范围为3至14.5岁(平均10.3岁)。男女比例为2.3:1。随访时间为1至17年(平均5年)。36%的患者曾接触过可能的病因:退烧药、牙皂、杀虫剂、苯、氯霉素和油漆。出现的症状为出血和贫血占72%,发热伴出血或贫血占28%。常见的出血部位为紫癜、鼻出血、牙龈和牙齿以及胃肠道。所有患者均接受了适当的支持治疗以及睾酮联合泼尼松龙治疗。对80例随访至死亡或诊断后至少一年的患者进行了治疗结果评估。三分之二的患者实验室检查结果类似严重再生障碍性贫血;死亡率为50%(40/80)。他们中的大多数在诊断后的前6个月死于感染或出血。恢复率为50%,完全缓解、部分缓解和初期缓解分别占28%、17%和5%。晚期恶性肿瘤发生率为2%。对于严重再生障碍性贫血或预后因素较差的患者,应考虑早期进行骨髓移植或给予抗淋巴细胞球蛋白治疗。

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