TY - JOUR
T1 - Monitoring therapy during treatment of von Willebrand Disease
AU - Favaloro, Emmanuel J
AU - Pasalic, Leonardo
AU - Curnow, Jennifer
N1 - Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.
PY - 2017/4
Y1 - 2017/4
N2 - von Willebrand factor (VWF) is an adhesive plasma protein that primarily acts to bridge platelets to sites of vascular injury and thus prevent bleeding. von Willebrand disease (VWD) is the most common inherited bleeding disorder and is caused by deficiency and/or defects of VWF, leading to low levels of plasma VWF or dysfunctional VWF. Factor VIII (FVIII) is also reduced in many patients with VWD, since VWF stabilizes and protects FVIII from degradation. Treatment of VWD primarily entails replacement of VWF, and sometimes FVIII, to protect against bleeding. This may entail use of VWF/FVIII concentrates, and/or desmopressin (1-deamino-8-d-arginine vasopressin) to release endogenous VWF in some patients. Adjunct therapies include antifibrinolytics and hormonal therapies in women. Optimal treatment of VWD entails measuring the effects of treatment, either as a trial before surgery or during therapeutic management. This is usually accomplished by performance of the same tests that are used to help diagnose VWD, although additional monitoring (clinically and/or by laboratory testing) may also be performed. The current review provides an overview of the treatment of VWD but is primarily focused on the monitoring of such therapy.
AB - von Willebrand factor (VWF) is an adhesive plasma protein that primarily acts to bridge platelets to sites of vascular injury and thus prevent bleeding. von Willebrand disease (VWD) is the most common inherited bleeding disorder and is caused by deficiency and/or defects of VWF, leading to low levels of plasma VWF or dysfunctional VWF. Factor VIII (FVIII) is also reduced in many patients with VWD, since VWF stabilizes and protects FVIII from degradation. Treatment of VWD primarily entails replacement of VWF, and sometimes FVIII, to protect against bleeding. This may entail use of VWF/FVIII concentrates, and/or desmopressin (1-deamino-8-d-arginine vasopressin) to release endogenous VWF in some patients. Adjunct therapies include antifibrinolytics and hormonal therapies in women. Optimal treatment of VWD entails measuring the effects of treatment, either as a trial before surgery or during therapeutic management. This is usually accomplished by performance of the same tests that are used to help diagnose VWD, although additional monitoring (clinically and/or by laboratory testing) may also be performed. The current review provides an overview of the treatment of VWD but is primarily focused on the monitoring of such therapy.
KW - Coagulants/therapeutic use
KW - Deamino Arginine Vasopressin/therapeutic use
KW - Drug Combinations
KW - Drug Monitoring/methods
KW - Factor VIII/therapeutic use
KW - Female
KW - Hemostatics/therapeutic use
KW - Humans
KW - Menorrhagia/complications
KW - Treatment Outcome
KW - von Willebrand Diseases/complications
KW - von Willebrand Factor/therapeutic use
U2 - 10.1055/s-0036-1585080
DO - 10.1055/s-0036-1585080
M3 - Review article
C2 - 27472426
SN - 0094-6176
VL - 43
SP - 338
EP - 354
JO - Seminars in Thrombosis and Hemostasis
JF - Seminars in Thrombosis and Hemostasis
IS - 3
ER -