The paradox of bleeding and thrombosis in thrombocythemia: is von Willebrand factor the link?
Thrombosis and bleeding, reflecting the extreme ends of hemostasis, are common complications in patients with thrombocythemia in its primary form or in association with polycythemia vera. The pathogenesis of these thrombohemorrhagic predispositions is not clearly understood. In the present study we describe the paradoxical and sequential occurrence of thrombotic and bleeding complications in six patients with thrombocythemia. Strikingly, in all patients some abnormality of plasma von Willebrand factor was detected. The potential role of von Willebrand factor in the pathogenesis of these hemostatic complications is discussed.
van Genderen
P J
PJ
Department of Internal Medicine II and Hematology, Erasmus University Dijkzigt, Rotterdam, The Netherlands.
Leenknegt
H
H
Michiels
J J
JJ
D000368:Aged; D000477:Alkylating Agents / Q000627:therapeutic use; D001241:Aspirin / Q000627:therapeutic use; D004916:Erythromelalgia / Q000188:drug therapy / Q000209:etiology; D005260:Female; D005343:Fibrinolytic Agents / Q000627:therapeutic use; D006470:Hemorrhage / Q000097:blood / Q000209:etiology*; D006474:Hemorrhagic Disorders / Q000097:blood / Q000209:etiology; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D002546:Ischemic Attack, Transient / Q000188:drug therapy / Q000209:etiology; D008297:Male; D008875:Middle Aged; D013920:Thrombocythemia, Essential / Q000097:blood / Q000150:complications* / Q000188:drug therapy; D019851:Thrombophilia / Q000097:blood / Q000209:etiology; D013927:Thrombosis / Q000097:blood / Q000209:etiology*; D014842:von Willebrand Diseases / Q000209:etiology* / Q000503:physiopathology; D014841:von Willebrand Factor / Q000502:physiology*
D000477:Alkylating Agents; D005343:Fibrinolytic Agents; D014841:von Willebrand Factor; D001241:Aspirin; D006918:Hydroxyurea