Thrombosis in Paroxysmal Nocturnal Hemoglobinuria - insights into the role of complement in thrombosis.

نویسنده

  • Ilene C Weitz
چکیده

s 2008;112:407.

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Intestinal perforation in a patient with paroxysmal nocturnal hemoglobinuria

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Mechanisms and clinical implications of thrombosis in paroxysmal nocturnal hemoglobinuria.

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disease characterized by a clone of blood cells lacking glycosyl phosphatidylinositol (GPI)-anchored proteins at the cell membrane. Deficiency of the GPI-anchored complement inhibitors CD55 and CD59 on erythrocytes leads to intravascular hemolysis upon complement activation. Apart from hemolysis, another prominent feature is a highly ...

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The most frequent and feared complication of paroxysmal nocturnal hemoglobinuria (PNH) is thrombosis. Recent research has demonstrated that the complement and coagulation systems are closely integrated with each influencing the activity of the other to the extent that thrombin itself has recently been shown to activate the alternative pathway of complement. This may explain some of the complexi...

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عنوان ژورنال:
  • Thrombosis research

دوره 125 Suppl 2  شماره 

صفحات  -

تاریخ انتشار 2010