Thalassemia intermedia: Revisited
Thalassemia intermedia: Revisited
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DOI：
10.1016/j.bcmd.2006.04.005
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发表时间：
2006-07-01
影响因子：
2.3
通讯作者：
Cappellini, Maria D.
中科院分区：
文献类型：
--
作者：
Taher, Ali;Hussain, Isma'eel;Cappellini, Maria D.
中间型地中海贫血涵盖广泛的临床β-地中海贫血表型。一些中间型地中海贫血患者直到成年才出现症状，而另一些患者从 2 岁时就出现症状。许多通常与中间型地中海贫血相关的临床并发症在重型地中海贫血中很少见，包括髓外造血、腿部溃疡、胆结石和血栓形成倾向。预防这些并发症（可能通过输血疗法）是理想的选择，因为它们可能难以控制。目前，许多中间型地中海贫血患者仅偶尔接受输血或不接受输血，因为他们能够将血红蛋白水平维持在 7-9 g/dl 之间；铁过载的风险（需要充分的螯合疗法）也是一个促成因素。目前，对于中间型地中海贫血患者开始和维持输血以预防或治疗并发症尚无明确的指南。在这里，我们回顾了中间型地中海贫血的主要临床并发症，并根据回顾性临床观察提出了一些治疗策略。 (c) 2006 Elsevier Inc. 保留所有权利。
Thalassemia intermedia encompasses a wide clinical spectrum of beta-thalassemia phenotypes. Some thalassemia intermedia patients are asymptomatic until adult life, whereas others are symptomatic from as young as 2 years of age. A number of clinical complications commonly associated with thalassemia intermedia are rarely seen in thalassemia major, including extramedullary hematopoiesis, leg ulcers, gallstones and thrombophilia. Prevention of these complications, possibly with blood transfusion therapy, is ideal since they may be difficult to manage. Currently, many patients with thalassemia intermedia receive only occasional or no transfusions, since they are able to maintain hemoglobin levels between 7-9 g/dl; the risk of iron overload, necessitating adequate chelation therapy, is also a contributing factor. At present, there are no clear guidelines for initiating and maintaining transfusions in thalassemia intermedia for the prevention or treatment of complications. Here, we review the major clinical complications in thalassemia intermedia and suggest some therapeutic strategies based on retrospective clinical observations. (c) 2006 Elsevier Inc. All rights reserved.