In the last three decades, with regular red cell transfusions and iron chelators, the survivals of patients with thalassemia, especially for TDT, have significantly improved. The most distinctive change was the
The intraction of alpha thalassemia with hetrozygus beta thalassemia. Br J Haematol. 1982;52(3):465–73.Kanavakis E, Wainscoat JS, Wood WG, et al. The interaction of alpha thalassaemia with heterozygous beta thalassaemia. Br J Haematol. 1982; 52 :465–473....
Thus alpha thalassemias are the result of decreased (α+) or absent (α0) production of alpha globin chains, and beta thalassemias are likewise the result of decreased (β+) or absent (β0) production of beta globin chains (Muncie and Campbell 2009; Cunningham 2010). The thalassemias are...
二、病因、发病机制 16号染色体的α基因突变(以缺失常见),形成异常的血红蛋白。 三、临床表现 四、诊断 1.血常规:Hb↓、MCV↓、MCH↓ 3.外周血涂片 镜检:低色素小细胞,如"靶型红细胞、高尔夫球型红细胞", 2.血红蛋白电泳 3.产前基因检测(绒毛膜绒毛取样、羊膜穿刺) 五、治疗 1.铁螯合剂:使铁通过粪/尿排...
The thalassemias are a group of inherited hematologic disorders caused by defects in the synthesis of one or more of the hemoglobin chains. Alphathalassemia is caused by reduced or absent synthesis of alpha globin chains, and beta thalassemia is caused by reduced or absent synthesis of beta globi...
The meaning of ALPHA-THALASSEMIA is thalassemia in which the hemoglobin chains designated alpha are affected.
The alpha thalassemia (α-thalassemia) syndromes are a group of hereditary anemias of varying clinical severity. They are characterized by reduced or absent production of 1 or more of the globin chains of which human hemoglobin is composed.
Jaundice and alpha gene triplication in beta-thalassemia: association or causation? Hematology. pr;11(2):109-12.Panigrahi I, Mahapatra M, Kumar R, Cahudhary VP, Sexsena R (2006) Jaundice and α-gene triplication in β-thalassemia: association or causation? Hematology 11:109–112...
We describe the haematological data and molecular results of a native family from Cádiz in that one is produced the a within heterozygous beta 0 thalassaemia (IVS-1, nt 1-G-->A), heterozygous alpha+ thalassaemia (-alpha 3.7) and alpha gene triplication (alpha alpha alpha 3.7). PATIENTS A...
alpha-thalassemia has been introduced to North America, North Europe, and Australia. Alpha-thalassemia is very heterogeneous at a clinical and molecular level. Four clinical conditions of increased severity are recognized: the silentcarrier state, the alpha-thalassemia trait, the intermediate form ofhem...