CAJ | 학술논문

目的探讨自体外周造血干细胞移植治疗成人皮肌炎的疗效.方法 1例21岁皮肌炎患者接受自体外周造血干细胞移植,随访6年.采用重组人粒细胞集落刺激因子动员外周造血干细胞后,予以环磷酰胺、甲泼尼龙、环孢素预处理,干细胞回输第3天开始使用兔抗人T淋巴细胞免疫球蛋白.观察症状体征、生化指标、造血重建及免疫恢复情况.结果出院时皮疹明显好转,后逐渐消退;四肢肌力由移植前Ⅳ级转为Ⅴ级并持续至今;移植后肌酸激酶显著下降,但又逐渐恢复到移植前水平;回输当天开始出现白细胞下降,第8天恢复正常造血功能.移植前后免疫功能均正常.结论对复杂难治性重症皮肌炎,可选择白体外周造血干细胞移植治疗.
목적 탐토자체외주조혈간세포이식치료성인피기염적료효.방법 1례21세피기염환자접수자체외주조혈간세포이식,수방6년.채용중조인립세포집락자격인자동원외주조혈간세포후,여이배린선알、갑발니룡、배포소예처리,간세포회수제3천개시사용토항인T림파세포면역구단백.관찰증상체정、생화지표、조혈중건급면역회복정황.결과 출원시피진명현호전,후축점소퇴;사지기력유이식전Ⅳ급전위Ⅴ급병지속지금;이식후기산격매현저하강,단우축점회복도이식전수평;회수당천개시출현백세포하강,제8천회복정상조혈공능.이식전후면역공능균정상.결론 대복잡난치성중증피기염,가선택백체외주조혈간세포이식치료.
Objective To investigate the efficacy of autologous peripheral hematopoietic stem cell transplantation in the treatment of adult dermatomyositis. Methods A 21-year-old patient with dermatomyositis received autologous peripheral hematopoietic stem cell transplantation and was followed up for 6 years. Autologous peripheral hematopoietic stem cells were mobilized by recombinant human granulocyte colony stimulating factor (rhG-CSF) before the transplantation, and the conditioning regimens consisted of cyclophosphamide,methylprednisolone and cyclosporin. Rabbit anti-human T lymphocyte immunoglobulin began to be applied on day 3 after retransfer of stem cells. The improvement in symptoms, physical signs and biochemical indicators was observed, and hematopoietic restructuration and immunity resurrection were evaluated after the transplantation. Results After the transplantation, skin eruption greatly improved and gradually subsided. The muscle force of extremities restored from level Ⅳ before transplantation to level Ⅴ. The level of creatine kinase declined sharply after transplantation, but gradually returned to previous level. Leucocyte count began to decrease on the day of retransfer, and returned to the normal level on day 8. Immune function remained normal before and after the transplantation. Conclusion Autologous peripheral hematopoietic stem cell transplantation is an alternative treatment for severe and refractory dermatomyositis.