Kynaston J A, West N C, Reid M M
Department of Child Health, University of Newcastle upon Tyne, UK.
Eur J Pediatr. 1993 Apr;152(4):306-8. doi: 10.1007/BF01956739.
The incidence and current management of red cell aplasia in children was determined from a retrospective survey of haematologists and paediatricians in the northern health region of England over a 7-year period. Thirty-three children were diagnosed: 4 had Diamond Blackfan anaemia, 22 transient erythroblastopenia of childhood, and 7 parvovirus B19 aplasia, with annual incidences of 1, 5 and 2 per 1,000,000 children respectively. Many were over-investigated. Three with Diamond Blackfan anaemia were steroid responsive. One with transient erythroblastopenia was retrospectively diagnosed because anaemia did not recur after steroids were stopped. Transient erythroblastopenia is the most common single cause of red cell aplasia in immunocompetent children. Time, observation and bone marrow examination before steroid therapy are the ways to distinguish transient erythroblastopenia from Diamond Blackfan anaemia or leukaemia. Interpretation of red cell indices using age-related percentiles may reduce the number of inappropriate investigations of the anaemia, but is often unhelpful in distinguishing the various causes of red cell aplasia.
通过对英格兰北部健康区域的血液学家和儿科医生进行为期7年的回顾性调查,确定了儿童红细胞再生障碍的发病率和当前治疗方法。共诊断出33名儿童:4例为先天性纯红细胞再生障碍性贫血,22例为儿童暂时性红细胞生成减少,7例为细小病毒B19所致再生障碍,每年发病率分别为每100万儿童中有1例、5例和2例。许多患儿接受了过度检查。3例先天性纯红细胞再生障碍性贫血患儿对类固醇治疗有反应。1例儿童暂时性红细胞生成减少患儿经回顾性诊断,因为停用类固醇后贫血未复发。儿童暂时性红细胞生成减少是免疫功能正常儿童红细胞再生障碍最常见的单一原因。在使用类固醇治疗前,通过时间、观察和骨髓检查来区分儿童暂时性红细胞生成减少与先天性纯红细胞再生障碍性贫血或白血病。使用与年龄相关的百分位数来解释红细胞指数可能会减少对贫血进行不适当检查的次数,但在区分红细胞再生障碍的各种病因方面通常并无帮助。