Matched-related allogeneic stem cell transplantation in Saudi patients with Fanconi anemia: 10 year's experience

被引：17
|
作者：
Ayas, M. [1 ]
Al-Jefri, A. [1 ]
Al-Seraihi, A. [1 ]
Elkum, N. [2 ]
Al-Mahr, M. [1 ]
El-Solh, H. [1 ]
机构：
[1] King Faisal Specialist Hosp & Res Ctr, Dept Pediat Hematol Oncol, Sect Pediat Stem Cell Transplant, Riyadh 11211, Saudi Arabia
[2] King Faisal Specialist Hosp & Res Ctr, Dept Biostat & Epidemiol, Riyadh 11211, Saudi Arabia
来源：
BONE MARROW TRANSPLANTATION | 2008年 / 42卷 / Suppl 1期
关键词：
Fanconi anemia; SCT; anti-thymocyte globulin; cyclophosphamide;
D O I：
10.1038/bmt.2008.114
中图分类号：
Q6 [生物物理学];
学科分类号：
071011 ;
摘要：
Allogeneic SCT is curative for bone marrow failure in Fanconi anemia (FA) patients but the optimal conditioning regimen is undetermined. We report here our experience with 56 FA patients who underwent allogeneic matched related SCT. The conditioning regimen varied according to time of SCT and disease status at SCT; 22 patients (group A) received Cy 20 mg/kg, thoraco-abdominal radiation and antithymocyte globulins (ATG); and 34 patients (group B) received Cy 60 mg/kg and ATG. Median time to engraftment was similar (14 days) in both groups. Hemorrhagic cystitis was significantly more common in group B. Overall survival and event-free survival of all patients were 85 and 78.3% respectively. For groups A and B respectively, overall survival was 72.5 and 96.9% (P = 0.013); and event-free survival was 72.5 and 82.3% (P = 0.3). The use of the nonradiation Cy/ATG regimen in matched related SCT for FA patients offers better overall and event-free survival.
引用
收藏
页码：S45 / S48
页数：4
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