Department of Clinical and Experimental Medicine, Università di Ferrara, Ferrara, Italy.
Ann N Y Acad Sci. 2010 Aug;1202:214-20. doi: 10.1111/j.1749-6632.2010.05550.x.
The severity of thalassemia intermedia depends on the degree of imbalance between alpha and non-alpha chains as well as other genetic and environmental factors that modify the natural history of the disease. By definition, the patients spontaneously maintain hemoglobin at or above 7 g/dL, sometimes at the price of intense hyperplasia of the bone marrow that is in turn responsible for bone deformities, osteoporosis, and extramedullary erythropoietic masses that often characterize thalassemia intermedia. Transfusion may become necessary with advancing age, during infection and pregnancy, and when hypersplenism develops. Splenectomy is often needed. Iron overload in nontransfused patients is due to increased gastrointestinal absorption and involves mainly the liver. Complications affecting the lives of patients with thalassemia intermedia include pulmonary hypertension, leg ulcers, pseudoxanthoma elasticum, gallstones, hepatocellular carcinoma, and thromboembolic events.
中间型地中海贫血的严重程度取决于α和非α链之间的失衡程度，以及其他改变疾病自然史的遗传和环境因素。根据定义，患者的血红蛋白自然维持在 7g/dL 或以上，有时骨髓过度增生，导致骨骼畸形、骨质疏松症和骨髓外红细胞生成肿块，这些都是中间型地中海贫血的特征。随着年龄的增长、感染和怀孕时以及发生脾功能亢进时，可能需要输血。脾切除术通常是必要的。未输血患者的铁过载是由于胃肠道吸收增加引起的，主要涉及肝脏。影响中间型地中海贫血患者生活的并发症包括肺动脉高压、腿部溃疡、弹性假黄瘤、胆结石、肝细胞癌和血栓栓塞事件。
