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I型异常红细胞生成性贫血。30年随访

Type I dyserythropoietic anemia. A 30-year follow-up.

作者信息

Maeda K, Saeed S M, Rebuck J W, Monto R W

出版信息

Am J Clin Pathol. 1980 Mar;73(3):433-8. doi: 10.1093/ajcp/73.3.433.

Abstract

Dyserythropoietic anemias are extremely rare disorders which often are misdiagnosed as megaloblastic anemia, DiGuglielmo's syndrome, refractory sideroblastic anemia, paroxysmal nocturnal hemoglobinuria, or other hemolytic anemias. Dyserythropoietic anemia is now subclassified into four types, designated Types I through IV, depending upon light and ultrastructural morphology, erythrocyte immunology, and the course of the disease. This report details the findings in a case of Type I dyserythropoietic anemia that represented a puzzling case of anemia for over 30 years. Many modes of drug therapy, as well as splenectomy, were not helpful. Interestingly, the family history was non-contributory. A brief review of the literature dealing with dyserythropoietic anemia is included.

摘要

异常红细胞生成性贫血是极为罕见的疾病,常被误诊为巨幼细胞贫血、迪古列尔莫综合征、难治性铁粒幼细胞贫血、阵发性夜间血红蛋白尿或其他溶血性贫血。根据光镜和超微结构形态、红细胞免疫学及病程,异常红细胞生成性贫血现分为四种类型,即I型至IV型。本报告详细阐述了一例I型异常红细胞生成性贫血的病例发现,该病例30多年来一直是令人困惑的贫血病例。多种药物治疗方式以及脾切除术均无效果。有趣的是,家族史无相关线索。文中还包括了对异常红细胞生成性贫血相关文献的简要综述。

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