Diagnostic and Therapeutic Center of Children's Blood Disease, Blood Disease Hospital, Institute of Hematology, Chinese Academy of Medical Sciences, 288 Nanjing Road, Tianjin, China.
Int J Hematol. 2012 Jul;96(1):34-8. doi: 10.1007/s12185-012-1092-z. Epub 2012 May 6.
Distinguishing hypoplastic myelodysplastic syndrome from aplastic anemia (AA) is challenging. In the present study, Japanese and Chinese pediatric hematologists and pathologists conducted a joint review of bone marrow (BM) smears and trephine biopsies in 100 children with acquired BM failure syndrome, using the criteria proposed in the 2008 edition of the World Health Organization classification of hematopoietic and lymphoid tissues. The final consensus for the diagnoses of 100 children was AA in 29 patients, refractory cytopenia of childhood (RCC) in 58 patients, and refractory cytopenia with multilineage dysplasia (RCMD) in 13 patients. No significant differences between Japanese and Chinese children were found with regards to clinical and laboratory findings, or the distribution of diagnoses. Patients with RCC/RCMD showed milder disease severity and BM hypocellularity than those with AA. To establish the provisional entities for RCC, it is essential to prospectively compare the clinical outcomes between AA and RCC groups in a large number of patients.
鉴别低增生性骨髓增生异常综合征与再生障碍性贫血（AA）具有挑战性。在本研究中，日本和中国的儿科血液学家和病理学家使用 2008 年版世界卫生组织造血和淋巴组织分类标准，对 100 例获得性骨髓衰竭综合征患儿的骨髓（BM）涂片和环钻活检进行了联合审查。100 例患儿的最终诊断共识为 29 例 AA、58 例儿童难治性血细胞减少症（RCC）和 13 例难治性血细胞减少症伴多系发育异常（RCMD）。日本和中国儿童在临床和实验室发现或诊断分布方面无显著差异。RCC/RCMD 患者的疾病严重程度和 BM 细胞减少程度均轻于 AA 患者。为了确定 RCC 的暂定实体，有必要在大量患者中前瞻性比较 AA 和 RCC 组的临床结局。
