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[溶血性贫血与维生素B12缺乏症]

[Hemolytic anemias and vitamin B12 deficieny].

作者信息

Dietzfelbinger Hermann, Hubmann Max

机构信息

Hämato-onkologische Schwerpunktpraxis, Herrsching.

出版信息

Dtsch Med Wochenschr. 2015 Aug;140(17):1302-10; quiz 1311-2. doi: 10.1055/s-0041-103562. Epub 2015 Aug 25.

Abstract

Hemolytic anemias consist of corpuscular, immun-hemolytic and toxic hemolytic anemias. Within the group of corpuscular hemolytic anemias, except for the paroxysmal nocturnal hemoglobinuria (PNH), all symptoms are caused by underlying heredetiary disorders within the red blood cell membran (hereditary spherocytosis), deficiencies of red cell enzymes (G6PDH- and pyrovatkinase deficiency) or disorders in the hemoglobin molecule (thalassaemia and sickle cell disease). Immune-hemolytic anemias are acquired hemolytic anemias and hemolysis is caused by auto- or allo-antibodies which are directed against red blood cell antigens. They are classified as warm, cold, mixed type or drug-induced hemolytic anemia. Therapy consists of glucocorticoids and other immunsuppressive drugs. Pernicious anemia is the most important vitamin B12 deficiency disorder. Diagnosis relies on cobalamin deficiency and antibodies to intrinsic factor. The management should focus on a possibly life-long replacement treatment with cobalamin.

摘要

溶血性贫血包括红细胞性、免疫性溶血性和中毒性溶血性贫血。在红细胞性溶血性贫血组中,除阵发性夜间血红蛋白尿(PNH)外,所有症状均由红细胞膜内潜在的遗传性疾病(遗传性球形红细胞增多症)、红细胞酶缺乏(葡萄糖-6-磷酸脱氢酶和丙酮酸激酶缺乏)或血红蛋白分子疾病(地中海贫血和镰状细胞病)引起。免疫性溶血性贫血是获得性溶血性贫血,溶血由针对红细胞抗原的自身抗体或同种抗体引起。它们分为温抗体型、冷抗体型、混合型或药物诱导的溶血性贫血。治疗包括使用糖皮质激素和其他免疫抑制药物。恶性贫血是最重要的维生素B12缺乏症。诊断依赖于钴胺素缺乏和内因子抗体。治疗应侧重于可能终生的钴胺素替代治疗。

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