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Management of thalassemia : ウィキペディア英語版
Management of thalassemia

* Mild thalassemia : patients with thalassemia traits do not require medical or follow-up care after the initial diagnosis is made.〔
〕 Patients with β-thalassemia trait should be warned that their condition can be misdiagnosed for the common Iron deficiency anemia. They should eschew empirical use of Iron therapy; yet iron deficiency can develop during pregnancy or from chronic bleeding.〔
〕 Counseling is indicated in all persons with genetic disorders, especially when the family is at risk of a severe form of disease that may be prevented.
* Severe thalassemia : Patients with severe thalassemia require medical treatment. A blood transfusion regimen was the first measure effective in prolonging life.〔
==Medication==
Patients with thalassemia gradually accumulate high levels of iron (Fe) in their bodies. This build-up of iron may be due to the disease itself, from irregular hemoglobin not properly incorporating adequate iron into its structure, or it may be due to the many blood transfusions received by the patient. This overload of iron brings with it many biochemical complications.
Two key players involved in iron transport and storage in the body are ferritin and transferrin. Ferritin is a protein present within cells that binds to Fe (II) and stores it as Fe (III), releasing it into the blood whenever required. Transferrin is an iron-binding protein present in blood plasma; transferrin acts as a transporter, carrying iron through blood and providing cells with the metal through endocytosis. Transferrin is highly specific to iron (III), and binds to it with an equilibrium constant of 1023 M−1 at a pH of 7.4.
Thalassemia results in nontransferrin-bound iron being available in blood as all the transferrin becomes fully saturated. This free iron is toxic to the body since it catalyzes reactions that generate free hydroxyl radicals.〔>〕 These radicals may induce lipid peroxidation of organelles like lysosomes, mitochondria, and sarcoplasmic membranes. The resulting lipid peroxides may interact with other molecules to form cross links, and thus either cause these compounds to perform their functions poorly, or render them non-functional altogether.〔 This iron overload may be treated with chelation therapy. Deferoxamine, deferiprone and deferasirox are the three most widely used iron-chelating agents.

抄文引用元・出典: フリー百科事典『 ウィキペディア(Wikipedia)
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