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阵发性冷性血红蛋白尿

Paroxysmal cold hemoglobinuria.

作者信息

Shanbhag Satish, Spivak Jerry

机构信息

Division of Hematology, Department of Medicine, The Johns Hopkins University School of Medicine, Suite 4500, 301 building 4940 Eastern Ave, Baltimore, MD 21224, USA.

Division of Hematology, Department of Medicine, The Johns Hopkins University School of Medicine, Suite 4500, 301 building 4940 Eastern Ave, Baltimore, MD 21224, USA.

出版信息

Hematol Oncol Clin North Am. 2015 Jun;29(3):473-8. doi: 10.1016/j.hoc.2015.01.004. Epub 2015 Mar 7.

Abstract

Paroxysmal cold hemoglobinuria is a rare cause of autoimmune hemolytic anemia predominantly seen as an acute form in young children after viral illnesses and in a chronic form in some hematological malignancies and tertiary syphilis. It is a complement mediated intravascular hemolytic anemia associated with a biphasic antibody against the P antigen on red cells. The antibody attaches to red cells at colder temperatures and causes red cell lysis when blood recirculates to warmer parts of the body. Treatment is mainly supportive and with red cell transfusion, but immunosuppressive therapy may be effective in severe cases.

摘要

阵发性冷性血红蛋白尿是自身免疫性溶血性贫血的一种罕见病因,主要表现为幼儿在病毒感染后出现的急性形式,以及某些血液系统恶性肿瘤和三期梅毒中的慢性形式。它是一种补体介导的血管内溶血性贫血,与针对红细胞上P抗原的双相抗体相关。该抗体在较低温度下附着于红细胞,并在血液回流到身体较温暖部位时导致红细胞溶解。治疗主要是支持性的,包括红细胞输血,但在严重病例中免疫抑制治疗可能有效。

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