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Semin Thromb Hemost 2011; 37(3): 315-321
DOI: 10.1055/s-0031-1273095
© Thieme Medical Publishers

Thrombosis in Patients with Paroxysmal Nocturnal Hemoglobinuria

Ilene C. Weitz1
  • 1Jane Anne Nohl Division of Hematology and Center for the Study of Blood Diseases, University of Southern California-Keck School of Medicine, Los Angeles, California
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Publication History

Publication Date:
31 March 2011 (online)

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ABSTRACT

Paroxysmal nocturnal hemoglobinuria is a disorder associated with hemolysis, pancytopenia, and thrombosis due to the loss of the glycosylphosphatidylinositol (GPI) anchored complement regulatory proteins. The mechanism of thrombosis is multifactorial. Although intravascular hemolysis has been implicated as the etiology, the effect of complement on GPI anchor-deficient platelets, granulocytes, monocytes, and endothelial cells contributes significantly to the risk of thrombosis. Moreover, there appears to be an underlying inflammatory state that is linked to hemostatic activation that may induce thrombosis through a pathway independent of hemolysis.

KEYWORDS

PNH - hemolysis - thrombosis

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Ilene Ceil WeitzM.D. 

Associate Clinical Professor of Medicine, Jane Anne Nohl Division of Hematology, Keck-USC School of Medicine

Los Angeles, CA

Email: iweitz@usc.edu