Beta-thalassemia intermedia (TI) is associated with a variety of serious clinical complications that require proactive and comprehensive management. These include skeletal deformities and osteopenia, compensatory extramedullary hematopoiesis and tumor formation, progressive splenomegaly, a hypercoagulable state resulting in thromboembolic events and pulmonary hypertension, and increased gastrointestinal iron absorption that often results in nontransfusional iron overload and liver damage. Although TI is generally considered a non-transfusion-dependent thalassemia, transfusion therapy may be an important part of the comprehensive management of this disease. This review describes the current state of the art for medical management of TI, with particular focus on the roles of splenectomy, transfusion, and iron chelation therapy.

中文翻译：

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当代治疗β地中海贫血的方法。

中间β地中海贫血（TI）与各种严重的临床并发症相关，需要积极，全面的管理。这些疾病包括骨骼畸形和骨质减少，代偿性髓外造血和肿瘤形成，进行性脾肿大，导致血栓栓塞事件和肺动脉高压的高凝状态，以及胃肠道铁吸收增加，常常导致非输血性铁超负荷和肝损害。尽管TI通常被认为是非输血依赖型地中海贫血，但输血治疗可能是该疾病综合管理的重要组成部分。这篇综述描述了TI医疗管理的最新状态，特别着重于脾切除术，输血和铁螯合疗法的作用。