Thrombosis in Patients with Paroxysmal Nocturnal Hemoglobinuria

Ilene C Weitz

doi:10.1055/s-0031-1273095

Seminars in Thrombosis and Hemostasis, Table of Contents

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

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

Abstract

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

Full Text

References

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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