Tsukimoto I, Ohara A, Taguchi N, Miyazaki S, Akatsuka J, Konishi S, Nagao T, Nakahata T, Akabane T
1st Department of Pediatrics, Toho University School of Medicine.
Rinsho Ketsueki. 1991 Nov;32(11):1439-46.
As a general rule, diagnostic criteria of aplastic anemia in children are the same as adult criteria. However, blood counts of normal children show wide age-related variation, therefore we must establish a system of adjustment for diagnosis of aplastic anemia in children. The data of children with aplastic anemia visiting our institutes from 1966 to 1990 were evaluated for this study. RBC below 350 x 10(4)/microliters, WBC below 4,000/microliters or neutrophils below 1,500/microliters, platelets below 8 x 10(4)/microliters, reticulocytes below 4 x 10(4)/microliters and lymphocytes over 60% were seemed to satisfy the diagnostic criteria of aplastic anemia proposed by the Study Group of hemopoietic Disorders sponsored by the Ministry of Health and Welfare of Japan. Fifteen children (4.6%) did not meet these criteria and as such were diagnosed as atypical aplastic anemia. Thirteen of them were in a pre-aplastic state and developed typical aplastic anemia within 6 months to 8 years after the initial diagnosis. Clinical findings of these patients showed the decrease in number of megakaryocytes and committed stem cells in bone marrow. Three of these patients developed acute non-lymphocytic leukemia, and 2 of them were diagnosed as Fanconi's anemias.
一般来说，儿童再生障碍性贫血的诊断标准与成人相同。然而，正常儿童的血细胞计数显示出与年龄相关的广泛差异，因此我们必须建立一个儿童再生障碍性贫血诊断的调整系统。本研究评估了1966年至1990年期间到我们研究所就诊的再生障碍性贫血患儿的数据。红细胞低于350×10⁴/微升、白细胞低于4000/微升或中性粒细胞低于1500/微升、血小板低于8×10⁴/微升、网织红细胞低于4×10⁴/微升以及淋巴细胞超过60%似乎符合日本厚生省赞助的造血障碍研究组提出的再生障碍性贫血诊断标准。15名儿童（4.6%）不符合这些标准，因此被诊断为非典型再生障碍性贫血。其中13名处于再生障碍前期，在初次诊断后6个月至8年内发展为典型再生障碍性贫血。这些患者的临床检查结果显示骨髓中巨核细胞和定向干细胞数量减少。其中3名患者发展为急性非淋巴细胞白血病，2名被诊断为范可尼贫血。
