Abstract: Sickle cell anemia is a autosomal dominant inheritance hemoglobin disease, caused by the replacement of Glu by Val in the 6th amino acid in beta chain of hemoglobin. Its clinical manifestations include chronic hemolytic anemia, infection, recurrence of pain crisis and histologic lesion caused by chronic ischemia. This article introduces the epidemiology, pathogenesy, clinical manifestation, diagnosis and treatment of sickle cell anemia, especially its hemodynamics effects. It provides a new way to the prevention and treatment of stroke for sickle cell anemia patients.

Key words: Sickle cell anemia; Transonography; Doppler; transcranial; Stroke

摘要： 镰状细胞贫血是一种常染色体显性遗传血红蛋白病，是因为血红蛋白β链第6位氨基酸谷氨酸被缬氨酸所代替，形成了异常的血红蛋白S，取代了正常血红蛋白。临床表现为慢性溶血性贫血、易感染和再发性疼痛危象以及慢性局部缺血导致的器官组织损害。本文介绍了镰状细胞贫血的流行病学、发病机制、临床表现、诊断及治疗措施，尤其对镰状细胞贫血对血液动力学的影响作了重点讨论，为该类患者的卒中预防和治疗提供了新途径。

关键词: 镰状细胞贫血; 多普勒; 经颅; 卒中