Pulmonary hypertension associated with thalassemia syndromes.
Pulmonary hypertension associated with thalassemia syndromes.
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DOI：
10.1111/nyas.13037
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发表时间：
2016-03
影响因子：
5.2
通讯作者：
Machado RF
中科院分区：
文献类型：
--
作者：
Fraidenburg DR;Machado RF
慢性溶血性贫血越来越被认为是肺动脉高压发展的重要危险因素。在地中海贫血综合征中，肺动脉高压的发生有多种机制，既有不同的，也有重叠的，并且在β-地中海贫血重度或中度患者中有所不同。重度β-地中海贫血的肺动脉高压与溶血的严重程度相关，但在慢性输血治疗良好的患者中，肺动脉高压的发展可能与心功能障碍和随后的铁超载毒性作用有关，而不是溶血。另一方面，β-地中海贫血中，由于溶血水平较低，存在多年而不需要频繁输血，因此肺动脉高压的发生率较高，而脾切除术在这两种类型中都起重要作用。标准治疗如慢性输血已被证明可以减轻肺动脉高压，适当的螯合治疗可以避免铁超载的毒性作用，但在许多患者中并不适用。使用肺血管扩张剂或其他疗法（如左旋肉碱）治疗地中海贫血相关肺动脉高压的证据有限。本文综述了地中海贫血综合征肺动脉高压的发病机制、流行病学、表现、诊断和治疗等方面的最新发现。
Chronic hemolytic anemia has increasingly been identified as an important risk factor for the development of pulmonary hypertension. Within the thalassemia syndromes, there are multiple mechanisms, both distinct and overlapping, by which pulmonary hypertension develops and that differ among β-thalassemia major or intermedia patients. Pulmonary hypertension in β-thalassemia major correlates with the severity of hemolysis, yet in patients whose disease is well treated with chronic transfusion therapy, the development of pulmonary hypertension can be related to cardiac dysfunction and the subsequent toxic effects of iron overload rather than hemolysis. β-thalassemia intermedia, on the other hand, has a higher incidence of pulmonary hypertension owing to the low level of hemolysis that exists over years without the requirement for frequent transfusions, while splenectomy is shown to play an important role in both types. Standard therapies such as chronic transfusion have been shown to mitigate pulmonary hypertension, and appropriate chelation therapy can avoid the toxic effects of iron overload, yet is not indicated in many patients. Limited evidence exists for the use of pulmonary vasodilators or other therapies, such as l-carnitine, to treat pulmonary hypertension associated with thalassemia. Here we review the most recent findings regarding the pathogenic mechanisms, epidemiology, presentation, diagnosis, and treatment of pulmonary hypertension in thalassemia syndromes.