Abnormalities of the erythrocyte membrane.
Abnormalities of the erythrocyte membrane.
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DOI：
10.1016/j.pcl.2013.09.001
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发表时间：
2013-12
影响因子：
2.6
通讯作者：
Gallagher, Patrick G.
中科院分区：
文献类型：
--
作者：
Gallagher, Patrick G.
关键词：
红细胞膜的原发性异常，包括遗传性球形红细胞增多症和遗传性椭圆形红细胞增多综合征，是遗传性溶血性贫血的一个重要类型。根据外周血涂片上的独特形态学分类，这些疾病的特征在于临床、实验室和遗传异质性。在这一组中，遗传性球形红细胞增多症患者更有可能经历症状性贫血。脾切除术治疗遗传性球形红细胞增多症对大多数患者是有效的。一旦被认为是常规的，脾切除术的长期风险，包括心血管疾病，血栓性疾病，肺动脉高压，以及青霉素耐药肺炎球菌的出现，在压倒性的脾切除术后感染的关注，导致重新评估的作用脾切除术的认识。目前的管理指南承认这些重要的考虑时，娱乐脾切除术，并建议医疗保健提供者，病人和家庭之间的详细讨论。遗传性椭圆形红细胞增多症是最常见的原发性红细胞膜蛋白异常。然而，大多数椭圆形红细胞增多症患者是无症状的，不需要治疗。
Primary abnormalities of the erythrocyte membrane, including the hereditary spherocytosis and hereditary elliptocytosis syndromes, are an important group of inherited hemolytic anemias. Classified by distinctive morphology on peripheral blood smear, these disorders are characterized by clinical, laboratory, and genetic heterogeneity. Among this group, hereditary spherocytosis patients are more likely to experience symptomatic anemia. Treatment of hereditary spherocytosis with splenectomy is curative in most patients. Once considered routine, growing recognition of the longterm risks of splenectomy, including cardiovascular disease, thrombotic disorders, and pulmonary hypertension, as well as the emergence of penicillin-resistant pneumococci, a concern for infection in overwhelming postsplenectomy infection, have led to re-evaluation of the role of splenectomy. Current management guidelines acknowledge these important considerations when entertaining splenectomy and recommend detailed discussion between health care providers, patient, and family. The hereditary elliptocytosis syndromes are the most common primary disorders of erythrocyte membrane proteins. However, most elliptocytosis patients are asymptomatic and do not require therapy.
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影响因子：
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