von Willebrand disease: recent advances in pathophysiology and treatment.
This review focuses on new developments in the pathophysiology and treatment of von Willebrand disease (vWd). New aspects of the cell biology, gene control, and structure-function correlates of von Willebrand factor (vWf) are reviewed. vWd is more prevalent than previously recognized, affecting up to 1% of the population; this is particularly evident in women's health. Blood group is an important determinant of von Willebrand factor levels; individuals of blood group O tend to have lower plasma levels of vWf than those in other blood groups. Currently available blood tests of vWf quantity and function are discussed, in addition to newer tests undergoing validation. Treatment of classical vWd with desmopressin acetate and plasma derivatives is discussed, as is the potential for intravenous immunoglobulin and corticosteroids in acquired vWd. Special situations, such as the management of vWd in pregnancy, are also discussed.
Phillips
M D
MD
Centeon, L.L.C., King of Prussia, Pennsylvania 19406-1310, USA. phillmd@centeon.com
Santhouse
A
A
D000017:ABO Blood-Group System; D000941:Antigens / Q000097:blood; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D005260:Female; D006801:Humans; D011247:Pregnancy; D011248:Pregnancy Complications; D013329:Structure-Activity Relationship; D014842:von Willebrand Diseases / Q000145:classification / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000235:genetics / Q000503:physiopathology* / Q000628:therapy*; D014841:von Willebrand Factor / Q000737:chemistry / Q000235:genetics / Q000502:physiology
D000017:ABO Blood-Group System; D000941:Antigens; C029417:Von Willebrand antigen; D014841:von Willebrand Factor; D003894:Deamino Arginine Vasopressin