Ramzi Mani, Nourani Habib, Zakerinia Maryam, Dehghani Mehdi, Vojdani Reza, Haghshenas Mansour
Department of Hematology, Shiraz University of Medical Sciences, Shiraz, Iran.
Exp Clin Transplant. 2010 Mar;8(1):61-5.
Over the past 2 decades, hematopoietic stem cell transplant has evolved from an experimental procedure to the standard of care, and it is integrated into the management of many diseases. Hematopoietic stem cell transplant was established at Shiraz University of Medical Sciences in 1993. Here, we describe 15 years experience with stem cell transplant at our center in southern Iran. We provide information on indication, donor type, conditioning chemotherapy regimen, outcome, survival, and long-term follow-up in our stem cell activity.
From May 1993 to October 2008, 423 patients underwent allogeneic (n=311) and autologous (n=112) stem cell transplants at our center. For allogeneic stem cell transplant, the conditioning chemotherapy regimen comprised busulfan, cyclophosphamide, and antithymocyte globulin for thalassemic patients; busulfan and cyclophosphamide for leukemia patients; and cyclophosphamide and antithymocyte globulin for patients with aplastic anemia.
During this period, 155 B-thalassemia major patients (mean age, 9.5 years; range, 2-20 years) underwent allogeneic marrow transplant. Of 155 patients with a diagnosis of thalassemia major, 112 are alive (72%) with full engraftment after a median follow-up of about 8.1 years (range, 12-184 months). During this time, 127 leukemia patients including acute myelogenous leukemia (n=68), acute lymphoblastic leukemia (n=30) and chronic myelogenous leukemia (n=29), received allogeneic stem cell transplant. In this group, long-term, disease-free survival (cure rate) was 67%, 60%, and 62%.
These data reflect the important role of hematopoietic stem cell transplant in improving survival for a variety of hematopoietic system disorders at our center in Southern Iran. In patients with B-thalassemia major hematopoietic stem cell transplant seems to be the treatment of choice, because it leads to a cure in all classes (Lucarelli risk group, I-III). Based on high success rates in patients with class II and III thalassemia with the addition of the antithymocyte globulin to conditioning regimen of stem cell transplant, we also recommend using this new method of conditioning in transplant of thalassemia patients.
在过去20年中，造血干细胞移植已从一种实验性手术发展成为标准治疗方法，并被纳入多种疾病的治疗中。1993年设拉子医科大学开展了造血干细胞移植。在此，我们描述了伊朗南部我们中心15年的干细胞移植经验。我们提供了有关干细胞移植活动中的适应症、供体类型、预处理化疗方案、结果、生存率和长期随访的信息。
1993年5月至2008年10月，我们中心有423例患者接受了异基因（n = 311）和自体（n = 112）干细胞移植。对于异基因干细胞移植，地中海贫血患者的预处理化疗方案包括白消安、环磷酰胺和抗胸腺细胞球蛋白；白血病患者为白消安和环磷酰胺；再生障碍性贫血患者为环磷酰胺和抗胸腺细胞球蛋白。
在此期间，155例重型β地中海贫血患者（平均年龄9.5岁；范围2 - 20岁）接受了异基因骨髓移植。在155例确诊为重型地中海贫血的患者中，112例存活（72%），在中位随访约8.1年（范围12 - 184个月）后实现完全植入。在此期间，127例白血病患者，包括急性髓性白血病（n = 68）、急性淋巴细胞白血病（n = 30）和慢性髓性白血病（n = 29），接受了异基因干细胞移植。在该组中，长期无病生存率（治愈率）分别为67%、60%和62%。
这些数据反映了造血干细胞移植在提高伊朗南部我们中心各种造血系统疾病生存率方面的重要作用。对于重型β地中海贫血患者，造血干细胞移植似乎是首选治疗方法，因为它能治愈所有类型（卢卡雷利风险组，I - III）。基于在II类和III类地中海贫血患者中，在干细胞移植预处理方案中添加抗胸腺细胞球蛋白取得的高成功率，我们还建议在移植地中海贫血患者时采用这种新的预处理方法。
