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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 (syndrome) is a group of hereditary heterogeneous diseases characterized by disorders in the synthesis of globin chains, which vary widely in clinical manifestations due to different genotypes and phenotypes. Thalassemia is divided into transfusion dependent and non-transfusion dependent thalassemia (NTDT) based on the need for regular blood transfusion. Patients with NTDT should have strict blood transfusion indications, and usually do not need blood transfusions when Hb≥50 g / L. However, blood transfusions may be needed during pregnancy and severe infections such as growth retardation, skeletal deformity, and splenomegaly. Complications should be considered when transfusion.