Lukarelli G, Galimberti M, Pol'ki P, Andzheluchchi E, Baronchiani D, Dzhardini K, Duratstsi S, Agostinelli F, Dzhordzhi K, Donati M
Gematol Transfuziol. 1993 Sep-Oct;38(8):7-11.
Thalassemia major is a progressive disease. Modern therapy with transfusions and iron chelation has greatly improved both the quality and length of thalassemics life. Nevertheless, progressive overload of iron in the organs, hepatitis and other infections acquired randomly imply the risk of mortality that increases with age. Bone marrow transplant from an HLA identical donor (a possibility for about 45%) represents the only form of radical cure of the disease. With the classification of the classes of risk we know today that a thalassemic child has a chance to die from transplant 3 to 100 with probability of being cured 94%. Thalassemics that receive the transplant at a more advanced stage of complications of the disease have a major risk of death due to the transplant of class 2 (14% probability of death due to the transplant against 83% chance of being cured). Class 43 patients have 9 chances out of 100 to die of transplant and 60% probability of being cured of thalassemia.
重型地中海贫血是一种进行性疾病。现代输血和铁螯合疗法极大地改善了地中海贫血患者的生活质量和寿命。然而，器官中铁的渐进性超负荷、随机获得的肝炎和其他感染意味着死亡风险会随着年龄增长而增加。来自人类白细胞抗原（HLA）匹配供体的骨髓移植（约45%的可能性）是该疾病唯一的根治方法。根据我们如今所了解的风险分级，一名患地中海贫血的儿童移植后有3%至100%的死亡几率，治愈概率为94%。在疾病并发症更严重阶段接受移植的地中海贫血患者，因2级移植而死亡的风险更高（移植导致死亡的概率为14%，而治愈的几率为83%）。43级患者移植后有9%的死亡几率，治愈地中海贫血的概率为60%。
