Ohba Rie, Usui Noriko, Ito Yuta, Yamauchi Hirofumi, Machishima Tomohito, Ishii Hiroto, Fukushima Ryoko, Yokoyama Hiroki, Shiota Yuko, Yahagi Yuichi, Yano Shingo, Dobashi Nobuaki, Aiba Keisuke
Department of Clinical Oncology and Hematology, The Jikei University Daisan Hospital.
Department of Clinical Oncology and Hematology, The Jikei University Hospital.
Rinsho Ketsueki. 2017;58(6):601-606. doi: 10.11406/rinketsu.58.601.
Myelodysplastic syndrome with myelofibrosis (MDS-F) is a disease with a poor prognosis, and patients with this condition are at an increased risk of engraftment failures after allogeneic hematopoietic stem cell transplantation (SCT). Azacitidine (AZA) is effective in high-risk MDS patients. However, the effects of AZA on MDS-F have not been elucidated. AZA was administered to a 62-year-old male with MDS-F for 7 days at a dose of 75 mg/m. Hematological improvements were observed after only 1 course of treatment. No suitable donor was found through the Japan Marrow Donor Program; therefore, the patient underwent umbilical cord blood transplant (UCBT). Neutrophil engraftment was observed on day 21 after the transplant procedure. He developed acute graft versus host disease (GVHD) of the skin (stage 3/grade II), but it could be controlled using prednisolone. Chronic GVHD was not observed and he was discharged in good general condition on day 68. While treatment prior to allogeneic SCT of MDS-F has not been established, in the present case, the hematological improvement brought about by AZA likely contributed to the patient's positive response to UCBT.
伴有骨髓纤维化的骨髓增生异常综合征（MDS-F）是一种预后较差的疾病，患有这种疾病的患者在异基因造血干细胞移植（SCT）后发生植入失败的风险增加。阿扎胞苷（AZA）对高危MDS患者有效。然而，AZA对MDS-F的影响尚未阐明。一名62岁患有MDS-F的男性患者接受了7天的AZA治疗，剂量为75mg/m²。仅经过1个疗程的治疗就观察到血液学改善。通过日本骨髓供者计划未找到合适的供者；因此，该患者接受了脐带血移植（UCBT）。在移植手术后第21天观察到中性粒细胞植入。他发生了皮肤急性移植物抗宿主病（GVHD）（3期/II级），但使用泼尼松龙可以控制。未观察到慢性GVHD，他在第68天状况良好地出院。虽然MDS-F异基因SCT前的治疗方法尚未确立，但在本病例中，AZA带来的血液学改善可能有助于患者对UCBT产生积极反应。
