Delfini C, Donati M, Marchionni D, Nesci S, Paradisi O, Valentini M, Nicolini G, Lucarelli G
Int J Cell Cloning. 1986 Jul;4(4):274-8. doi: 10.1002/stem.5530040405.
In order to evaluate the possibility of a patient with thalassemia finding an HLA-identical sibling donor, we performed an analysis of HLA antigens in families of thalassemic patients. The pattern distribution was not significantly different from the expected ratio 25:50:25. When the siblings were subdivided according to the age of the patients (under or over 5 years), the above pattern remained unmodified for both the age groups. The average size of the 129 thalassemic families was 2.4. Thus, taking into account that thalassemic patients have an average of 1.4 siblings and that the HLA genotype distribution is expected as such, HLA-matched siblings are available for 33% of the patients. Because an additional 8.5% of the patients were found HLA-phenotypically identical to one parent, the chance for a patient with thalassemia to find a suitable donor for bone marrow transplantation would be increased to 41.5%. Our preliminary data cannot be extrapolated to the overall Italian thalassemic population; however, it can be inferred that for a patient with thalassemia, the chance of finding a suitable donor for bone marrow transplantation is not reduced.
为了评估地中海贫血患者找到 HLA 完全相同的同胞供者的可能性，我们对地中海贫血患者家庭的 HLA 抗原进行了分析。其模式分布与预期比例 25:50:25 无显著差异。当根据患者年龄（5 岁以下或 5 岁以上）对同胞进行细分时，两个年龄组的上述模式均未改变。129 个地中海贫血家庭的平均规模为 2.4 人。因此，考虑到地中海贫血患者平均有 1.4 个兄弟姐妹，且 HLA 基因型分布预期如此，33%的患者可找到 HLA 匹配的同胞。由于另外 8.5%的患者被发现 HLA 表型与父母之一相同，地中海贫血患者找到合适的骨髓移植供者的机会将增加到 41.5%。我们的初步数据不能外推至意大利全体地中海贫血人群；然而，可以推断，对于地中海贫血患者来说，找到合适的骨髓移植供者的机会并未减少。
