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β thalassemia major (Mediterranean anemia or Cooley anemia) is caused by a β o /β o genotype. No functional β chains are produced, and thus no hemoglobin A can be assembled. This is the most severe form of β-thalassemia; β thalassemia intermedia is caused by a β + /β o or β + /β + genotype. In this form, some hemoglobin A is produced;
Treatment. Blood transfusion, possible splenectomy [ 1][ 4] Alpha-thalassemia ( α-thalassemia, α-thalassaemia) is a form of thalassemia involving the genes HBA1 [ 5] and HBA2. [ 6] Thalassemias are a group of inherited blood conditions which result in the impaired production of hemoglobin, the molecule that carries oxygen in the blood. [ 7]
The brown areas contain hemosiderin. Specialty. Hematology. Hemosiderosis is a form of iron overload disorder resulting in the accumulation of hemosiderin . Types include: Transfusion hemosiderosis [ 1] Idiopathic pulmonary hemosiderosis. Transfusional diabetes [ 2][ 3] Organs affected:
Beta-thalassemia; Other names: Microcytemia, beta type [1] Beta thalassemia genetics, the picture shows one example of how beta thalassemia is inherited. The beta globin gene is located on chromosome 11. A child inherits two beta globin genes (one from each parent). Specialty: Hematology: Types: Thalassemia minor, intermediate and major [2] Causes
Hemoglobinopathy is the medical term for a group of inherited blood disorders involving the hemoglobin, the protein of red blood cells. [ 1] They are single-gene disorders and, in most cases, they are inherited as autosomal co-dominant traits. [ 2]
Hemoglobin E ( HbE) is an abnormal hemoglobin with a single point mutation in the β chain. At position 26 there is a change in the amino acid, from glutamic acid to lysine (E26K). Hemoglobin E is very common among people of Southeast Asian, Northeast Indian, Sri Lankan and Bangladeshi descent. [ 1][ 2]
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Normal levels range from 2.1-3.2%, but in the beta-thalassemia disorder, the levels increase to 3.5-6.0%. Additionally, individuals with beta-thalassemia exhibit a high red cell count and low hemoglobin levels. [3] Individuals that express lower levels of hemoglobin A2, have the a 0-thalassemia trait or homozygous gene for a +-thalassemia. [2]