Residual hemopoiesis and myelodysplasia development in aplastic anemia children. -Epidemiologic and clinical observation

再生障碍性贫血儿童的残余造血和骨髓增生异常的发展。

• 批准号: 16591058
• 负责人: OHARA Akira
• 金额: $ 2.02万
• 依托单位: Toho University
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Scientific Research (C)
• 财政年份: 2004
• 资助国家: 日本
• 起止时间: 2004 至 2007
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-16591058/
• 关键词: bone marrow failure; aplastic anemia; myelodyndastic syndrome; 骨髄異形成症候群

Although both of an aplastic anemia (AA) and a myelodysplastic syndrome(MDS)-refractory anemia(RA) are bone marrow failure syndrome, disease-overlapping between these disease has been discussed. Aim of this study is clarified that etiology and clinical characteristics of MDS-developed AA patient and residual hemopoiesis in these bone marrow failure syndrome.According to database that has been established by Japanese Society of Pediatric Hematology, a prognosis of MDS developed AA patient depends on chromosome abnormalities. Trisomy 8 is good, however monosomy 7 is poor prognostic marker. Incidence of MDS development in RA patients was low. Clinical and hematological response for immunosuppressive therapy (IST) was suspected as high as 50-60% in both AA and MDS-RA. MCV was also high in both type of bone marrow failure because MCV reflects ineffective erythropoiesis.For the purpose of evaluation for residual hemopoiesis, multidimensional flow cytometry analysis (MDF) was introduced in a small-scale study. As a tentative result, MDF technique has a possibility to detect exhaustion of immature myeloid cells at an AA diagnosis and was able to follow hemopoiesis recovery after IST, and also detect aberrant Fells on abnormal maturation pathway.

尽管同性贫血（AA）和骨髓增生综合征（MDS） - 性疗法贫血（RA）都是骨髓衰竭综合征，但已经讨论了这些疾病之间的疾病过多。这项研究的目的阐明了MDS开发的AA患者的病因和临床特征和这些骨髓衰竭综合征中的残留造血。三体构造很好，但是单肌第7个是较差的预后标记。 RA患者MDS发育的发生率很低。 AA和MDS-RA的临床和血液学反应怀疑高达50-60％。 MCV在两种类型的骨髓衰竭中也很高，因为MCV反映了无效的红细胞生成。为了评估残留造血的目的，在一项小型研究中引入了多维流式细胞仪分析（MDF）。作为暂定的结果，MDF技术有可能在AA诊断时检测到未成熟的髓样细胞的精疲力尽，并且能够在IST后跟随造血恢复，并且还检测到异常成熟途径上的异常跌倒。

项目成果

ALL after one year remission by immunosuppressive therapy for aplastic anemia

再生障碍性贫血免疫抑制治疗缓解一年后的 ALL

• 发表时间: 2007
• 期刊: Jpn J Pediatr Hematol 21(516)
• 作者: Suzuki;T.;Mamada;Y.;Koda;K.;Murata;T.;Ohara;A.;Kojima;S.;Ishiguro;S
• 通讯作者: S

後天性再生不良性貧血に対する免疫抑制療法後の再発例の検討

获得性再生障碍性贫血免疫抑制治疗后复发病例调查

• 发表时间: 2007
• 作者: 小原 明;他9名
• 通讯作者: 他9名

Prospective randomized multicenter study comparing antithymocyte globulin (ATG) alone with ATG plus cyclosporine (CsA) for treatment of children with nonsevere aplastic anemia

比较单独抗胸腺细胞球蛋白 (ATG) 与 ATG 加环孢素 (CsA) 治疗儿童非严重再生障碍性贫血的前瞻性随机多中心研究

• 发表时间: 2005
• 作者: Yagasaki;H;et. al.
• 通讯作者: et. al.

Epidemiological survey of aplastic anemia children in Japan

日本儿童再生障碍性贫血流行病学调查

• 发表时间: 2007
• 作者: Ohara;A
• 通讯作者: A

Prospective randomized multicenter study comparing antithymocyte globulin(ATG)alone with ATG plus cyclosporine(CsA)for treatment of children with nonsevere aplastic anemia

比较单独抗胸腺细胞球蛋白（ATG）与 ATG 加环孢素（CsA）治疗儿童非严重再生障碍性贫血的前瞻性随机多中心研究

• 发表时间: 2005
• 作者: Yagasaki H;Ohara A;他13名
• 通讯作者: 他13名