Shibuya Atsushi, Ishii Saori, Obinata Kaoru
Department of Pediatrics, Saitama Medical School, 38 Morohongo Iruma-gun, Saitama, Japan 350-0495.
Hematology. 2002 Oct;7(5):301-4. doi: 10.1080/1024533021000037162.
A 9-year-old girl who had hepatitis-associated aplastic anemia was treated intermittently with methylprednisolone pulse therapy and growth factors (granulocyte-colony stimulating factor (G-CSF), recombinant human erythropoietin (rhEpo) and cyclosporin A (CyA) for over two years. At this time, there was hematological improvement, but chromosome analysis revealed monosomy 7. After six months, there was progression to myelodysplastic syndrome (MDS) (stage in refractory anemia of excess blasts (RAEB)) with monosomy 7, monosomy 6, marker chromosome and with hematological deterioration. She received bone marrow (1.57 x 10(5) cells kg(-1) (patient body weight)) plus cord blood cell (0.3 x 10(7) cells kg(-1) (patient body weight)) transplantation from her brother, 2 years and 7 months after the diagnosis of hepatitis-associated aplastic anemia. Engraftment was achieved after two weeks, and acute graft-versus-host disease occurred in a mild form after four weeks. Hematological remission has been continuous for 20 months after bone marrow transplantation. Transformation of hepatitis-associated aplastic anemia to MDS with the monosomy 7, monosomy 6 and marker chromosome in this patient was considered to have been related to the administration of high doses of immunosuppressive drugs plus growth factors.
一名患有肝炎相关性再生障碍性贫血的9岁女孩接受了两年多的甲泼尼龙冲击疗法及生长因子（粒细胞集落刺激因子（G-CSF）、重组人促红细胞生成素（rhEpo）和环孢素A（CyA））间歇性治疗。此时，血液学有改善，但染色体分析显示7号染色体单体。6个月后，病情进展为骨髓增生异常综合征（MDS）（难治性贫血伴原始细胞增多（RAEB）期），伴有7号染色体单体、6号染色体单体、标记染色体，且血液学恶化。在诊断为肝炎相关性再生障碍性贫血2年7个月后，她接受了来自其哥哥的骨髓（1.57×10⁵细胞·kg⁻¹（患者体重））加脐血细胞（0.3×10⁷细胞·kg⁻¹（患者体重））移植。两周后实现植入，四周后发生轻度急性移植物抗宿主病。骨髓移植后血液学缓解持续了20个月。该患者肝炎相关性再生障碍性贫血转化为伴有7号染色体单体、6号染色体单体和标记染色体的MDS被认为与高剂量免疫抑制药物加生长因子的使用有关。
