重型β地中海贫血的输血治疗研究进展

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Abstract Thalassemia is a serious public health problem in the world.In China,Guangdong and Guangxi are regions of high incidence.Severe β-thalassemia is an ineffective hematopoietic or hemolytic anemia resulting from the accumulation of α -globin chain imbalance caused by the decrease or absence of β -globin production,which is a serious fatal disease.From 3 to 6 months after birth,the patient began to develop chronic progressive hemolysis,accompanied by developmental retardation,pale complexion,hepatosplenomegaly and other symptoms.As the condition worsens,bone swelling occurs.Without blood transfusion,the patientundefineds life span is as little as 5 years.Although the life span of the patients with severe β-thalassemia is significantly prolonged after regular blood transfusion,repeated blood transfusion resulted in hypothyroidism,diabetes mellitus and hyposexual function due to iron overload.Standard ironremoving therapy can lighten the iron load of patients,but long-term iron removal seriously affects the quality of life of patients,so compliance is poor.In recent years,hematopoietic stem cell transplantation (HSCT)is the only medical method to cure severe β-thalassemia.The life quality of patients with thalassemia after stem cell transplantation is significantly higher than that of patients treated with traditional blood transfusion and iron removal,but there are still problems of life-threatening complications and high cost of treatment after transplantation.

Key words： &beta -thalassemia Severe Blood transfusion Complications Hematopoietic stem cell transplantation

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	WEI Hai-chun
	ZHENG Min

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WEI Hai-chun,ZHENG Min. Study progress of blood transfusion therapy for severe β-thalassaemia major[J]. 中国当代医药, 2018, 25(26): 31-35.

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http://dangdaiyiyao.com/EN/ OR http://dangdaiyiyao.com/EN/Y2018/V25/I26/31

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