非输血依赖性地中海贫血患者的输血治疗

Transfusion therapy in patients with non-transfusion dependent thalassemia

地中海贫血(综合征)是一组以珠蛋白链合成障碍为特征的遗传性异质性疾病,因变异基因型不同和表现型差异临床表现差别很大。根据是否需要定期输血可将地中海贫血分为输血依赖性和非输血依赖性地中海贫血(NTDT)。NTDT患者应严格掌握输血指征,通常Hb≥50 g/L时不需输血,但在妊娠和严重感染等应激状态时可能需要输血,在生长发育迟缓、骨骼畸形、脾脏明显增大等严重并发症时应考虑输血。

Thalassemia is a set of heterogeneous genetic disorders characterized by abnormalities of synthesis of globin chains. Its clinical manifestations vary widely because of different variant genotypes and various phenotypes. It can be divided into transfusion-dependent thalassemia（ TDT） and non-transfusion-dependent thalassemia（ NTDT） in accordance with the requirement of regular blood transfusions. NTDT is consisted of the following variants： beta thalassemia,alpha thalassemia of Mediterranean variant（ Hb H disease） and Hb E disease. Transfusion indications in patients with NTDT should be monitored strictly. They may not require transfusion in general when Hb≥50 g / L,while they may require occasional blood transfusions during infection,pregnancy or any other stressful settings. They may also require more frequent,yet temporary transfusions in the case of poor growth or development,skeletal deformities,rapid splenomegaly and other specific complications during childhood.