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[阵发性夜间血红蛋白尿:病理生理学与治疗管理]

[Paroxysmal nocturnal hemoglobinuria: physiopathology and therapeutic management].

作者信息

Altafulla M, Díaz R

出版信息

Rev Med Panama. 1989 Sep;14(3):145-53.

PMID:2682813
Abstract

The authors study the pathophysiology and the therapy of patients with paroxysmal nocturnal hemoglobinuria according with the literature reviewed. They also refer to have studied seven cases with HPN, two of which were women and five men, between the ages of 26 and 76 years of age, with symptomatology that varied from moderate, chronic hemolytic anemia and acholuria, to severe pancytopenia with bleeding, infections, and requiring transfusions, antibiotics, corticosteroids, androgens and other supportive measures. Most of them had enlargement of the spleen and liver, especially during hemolytic crises.

摘要

作者根据所综述的文献研究了阵发性夜间血红蛋白尿患者的病理生理学及治疗方法。他们还提到研究了7例阵发性夜间血红蛋白尿患者,其中2例为女性,5例为男性,年龄在26岁至76岁之间,症状从中度慢性溶血性贫血和无胆色素尿,到伴有出血、感染且需要输血、使用抗生素、皮质类固醇、雄激素及其他支持措施的严重全血细胞减少不等。他们中的大多数人有脾脏和肝脏肿大,尤其是在溶血危象期间。

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