{"title":"β-地中海贫血患者血栓栓塞事件和神经系统损害的研究进展","authors":"Negin Kheiri, Morteza Zangeneh Soroush, Marzieh Aboutorabi","doi":"10.18502/ijpho.v13i4.13773","DOIUrl":null,"url":null,"abstract":"Β-thalassemia is the severe form of genetic illnesses which decreases the hemoglobin synthesis. One of the major complications in thalassemic syndromes, including β-thalassemia major and intermedia is thromboembolic events. In addition, thromboembolic events are more common in non-transfusion-dependent thalassemia than those in well-transfusion-dependent β-thalassemia. A combination of hypercoagulable states including, abnormalities in red blood cells, and platelet, antithrombin III, protein C, and protein S, and splenectomy are involved in thrombotic events, but thromboembolic events can be prevented and treated in these patients via blood transfusion, hydroxyurea, anticoagulants, and aspirin. Moreover, recent studies have demonstrated the involvement of the brain lesion in β-thalassemia patients. The involvement of vascular events of brain in patients with β-thalassemia intermedia is 29-83%, but the rate of asymptomatic brain lesions in the healthy people is 0-11%. In addition, neurological complications which have been attributed to various factors are chronic hypoxia, iron overload, bone marrow expansion, and desferrioxamine neurotoxicity. This review evaluated thromboembolic events and neurological lesions in patients with β-thalassemia and its probable curative therapy.","PeriodicalId":44212,"journal":{"name":"Iranian Journal of Pediatric Hematology and Oncology","volume":null,"pages":null},"PeriodicalIF":0.4000,"publicationDate":"2023-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"A review on thromboembolic events and neurological lesions in patients with β-thalassemia\",\"authors\":\"Negin Kheiri, Morteza Zangeneh Soroush, Marzieh Aboutorabi\",\"doi\":\"10.18502/ijpho.v13i4.13773\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Β-thalassemia is the severe form of genetic illnesses which decreases the hemoglobin synthesis. One of the major complications in thalassemic syndromes, including β-thalassemia major and intermedia is thromboembolic events. In addition, thromboembolic events are more common in non-transfusion-dependent thalassemia than those in well-transfusion-dependent β-thalassemia. A combination of hypercoagulable states including, abnormalities in red blood cells, and platelet, antithrombin III, protein C, and protein S, and splenectomy are involved in thrombotic events, but thromboembolic events can be prevented and treated in these patients via blood transfusion, hydroxyurea, anticoagulants, and aspirin. Moreover, recent studies have demonstrated the involvement of the brain lesion in β-thalassemia patients. The involvement of vascular events of brain in patients with β-thalassemia intermedia is 29-83%, but the rate of asymptomatic brain lesions in the healthy people is 0-11%. In addition, neurological complications which have been attributed to various factors are chronic hypoxia, iron overload, bone marrow expansion, and desferrioxamine neurotoxicity. This review evaluated thromboembolic events and neurological lesions in patients with β-thalassemia and its probable curative therapy.\",\"PeriodicalId\":44212,\"journal\":{\"name\":\"Iranian Journal of Pediatric Hematology and Oncology\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":0.4000,\"publicationDate\":\"2023-10-08\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Iranian Journal of Pediatric Hematology and Oncology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.18502/ijpho.v13i4.13773\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"PEDIATRICS\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Iranian Journal of Pediatric Hematology and Oncology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.18502/ijpho.v13i4.13773","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"PEDIATRICS","Score":null,"Total":0}
A review on thromboembolic events and neurological lesions in patients with β-thalassemia
Β-thalassemia is the severe form of genetic illnesses which decreases the hemoglobin synthesis. One of the major complications in thalassemic syndromes, including β-thalassemia major and intermedia is thromboembolic events. In addition, thromboembolic events are more common in non-transfusion-dependent thalassemia than those in well-transfusion-dependent β-thalassemia. A combination of hypercoagulable states including, abnormalities in red blood cells, and platelet, antithrombin III, protein C, and protein S, and splenectomy are involved in thrombotic events, but thromboembolic events can be prevented and treated in these patients via blood transfusion, hydroxyurea, anticoagulants, and aspirin. Moreover, recent studies have demonstrated the involvement of the brain lesion in β-thalassemia patients. The involvement of vascular events of brain in patients with β-thalassemia intermedia is 29-83%, but the rate of asymptomatic brain lesions in the healthy people is 0-11%. In addition, neurological complications which have been attributed to various factors are chronic hypoxia, iron overload, bone marrow expansion, and desferrioxamine neurotoxicity. This review evaluated thromboembolic events and neurological lesions in patients with β-thalassemia and its probable curative therapy.