Hama Asahito
Department of Hematology and Oncology, Children's Medical Center, Japanese Red Cross Aichi Medical Center Nagoya First Hospital.
Rinsho Ketsueki. 2022;63(9):1035-1041. doi: 10.11406/rinketsu.63.1035.
In 2008, the World Health Organization proposed a provisional entity of childhood myelodysplastic syndrome (MDS) without a blasts increase, which was referred to as the refractory cytopenia of childhood (RCC). We performed a central review of bone marrow morphology in 252 children with acquired bone marrow failure syndromes to clarify the clinical relevance of the RCC. The RCC was divided two categories, namely, RCC without multilineage dysplasia (MLD) and RCC with MLD, which is similar to MDS with MLD in adult MDS. Furthermore, the clinical outcomes were investigated for cases diagnosed with aplastic anemia, RCC without MLD, and RCC with MLD. The response rates to immunosuppressive therapy and the incidence of the development of the new chromosomal aberration did not significantly differ among the three groups. The RCC with MLD can be adopted in childhood MDS since children with this condition exhibited a frequent chromosomal aberration at the time of diagnosis and a high frequency of secondary graft failure after a hematopoietic cell transplantation.
2008年，世界卫生组织提出了一种儿童骨髓增生异常综合征（MDS）的临时实体，其原始细胞无增加，被称为儿童难治性血细胞减少症（RCC）。我们对252例获得性骨髓衰竭综合征患儿的骨髓形态进行了集中审查，以阐明RCC的临床相关性。RCC分为两类，即无多系发育异常（MLD）的RCC和有MLD的RCC，后者类似于成人MDS中有MLD的MDS。此外，还对诊断为再生障碍性贫血、无MLD的RCC和有MLD的RCC的病例的临床结局进行了研究。三组之间免疫抑制治疗的缓解率和新发染色体异常的发生率没有显著差异。有MLD的RCC可用于儿童MDS，因为患有这种疾病的儿童在诊断时表现出频繁的染色体异常，并且在造血细胞移植后继发移植失败的频率很高。
