Hamidieh A A, Dehaghi M Ostadali, Paragomi P, Navaei S, Jalali A, Eslami G Ghazizadeh, Behfar M, Ghavamzadeh A
Hematology, Oncology and Stem Cell Transplantation Research Center, Tehran University of MedicalSciences, Tehran, Iran.
Bone Marrow Transplant. 2015 Apr;50(4):545-52. doi: 10.1038/bmt.2014.307. Epub 2015 Jan 26.
The best donors for hematopoietic SCT (HSCT) are fully-matched siblings. In patients without fully-matched siblings, HLA registries or cord blood banks are alternative strategies with some restrictions. Owing to the high rate of consanguineous marriage in our country, between 2006 and 2013, extended family searches were undertaken in Hematology-Oncology Research Center and Stem Cell Transplantation (HORCSCT), Tehran, Iran, in 523 HSCT candidates with parental consanguinity and no available HLA identical sibling. Fully-matched other-relative donors were found for 109 cases. We retrospectively studied the HSCT outcome in these patients. Median time to neutrophil engraftment was 13 days (range: 9-31days). In 83 patients, full chimerism and in 17 patients, mixed chimerism was achieved. Acute GvHD (aGvHD) grade II-IV appeared in 36 patients (33%). The frequency of aGvHD development in various familial subgroups was NS. Five patients expired before day+100. In the surviving 104 cases, chronic GvHD developed in 20 patients (19.2%). The distantly related subgroup had significantly a higher rate of cGvHD (P=0.04). The 2-year OS and disease-free survival (DFS) were 76.7±4.5% and 71.7±4.7%, respectively. No significant difference in OS (P=0.30) and DFS (P=0.80) was unraveled between various familial relationships. Our considerable rate of fully-matched non-sibling family members and the favorable outcome support the rationale for extended family search in regions where consanguineous marriage is widely practiced.
造血干细胞移植(HSCT)的最佳供者是全相合的同胞。对于没有全相合同胞的患者,HLA登记库或脐带血库是有一定限制的替代策略。由于我国近亲结婚率较高,2006年至2013年期间,伊朗德黑兰血液肿瘤研究中心及干细胞移植中心(HORCSCT)对523例有父母近亲关系且无HLA相合同胞的HSCT候选者进行了远亲搜索。为109例患者找到了全相合的其他亲属供者。我们对这些患者的HSCT结果进行了回顾性研究。中性粒细胞植入的中位时间为13天(范围:9 - 31天)。83例患者实现了完全嵌合,17例患者实现了混合嵌合。36例患者(33%)出现了II - IV级急性移植物抗宿主病(aGvHD)。不同家族亚组中aGvHD发生的频率无统计学意义。5例患者在+100天前死亡。在存活的104例患者中,20例(19.2%)发生了慢性移植物抗宿主病(cGvHD)。远亲亚组的cGvHD发生率显著更高(P = 0.04)。2年总生存率(OS)和无病生存率(DFS)分别为76.7±4.5%和71.7±4.7%。不同家族关系之间的OS(P = 0.30)和DFS(P = 0.80)无显著差异。我们相当比例的全相合非同胞家庭成员以及良好的结果支持了在近亲结婚普遍的地区进行远亲搜索的合理性。