Ghavamzadeh A, Alimoghadam K, Nasseri P, Jahani M, Khodabandeh A, Ghahremani G
Department of Oncology, Shariati Hospital, Tehran University of Medical Science, Iran.
Bone Marrow Transplant. 1999 Feb;23(3):299-301. doi: 10.1038/sj.bmt.1701567.
Dyskeratosis congenita is recognized by its dermal lesions and constitutional aplastic anemia in some cases. We report successful allogeneic bone marrow transplantation in two siblings with this disease from their sister, and their long term follow-up. We used reduced doses of cyclophosphamide and busulfan for conditioning instead of total body irradiation. Also, we report late adverse effects of transplantation which are not distinguishable from the natural course of disease.
先天性角化不良以其皮肤病变为特征，部分病例伴有体质性再生障碍性贫血。我们报告了两例患有该病的兄弟姐妹接受其姐姐的异基因骨髓移植成功及其长期随访情况。我们使用环磷酰胺和白消安的减量方案进行预处理，而非全身照射。此外，我们还报告了移植的晚期不良反应，这些反应与疾病的自然病程难以区分。
