Michiels et al classified causes for the acquired von Willebrand Syndrome (AvWS). Identifying the cause of AvWS is essential for determining the best therapy. The authors are from University Hospital Antwerp in Belgium.
With acquired von Willebrand disease:
(1) there is no evidence of familial von Willebrand disease
(2) the patient has a history of normal coagulation prior to the onset of some condition
(3) the acquired coagulopathy responds to treatment of the condition
The acquired von Willebrand Syndrome may be divided into immune and nonimmune causes.
Nonimmune Cause
Mechanism
Therapy
hypothyroidism
decreased synthesis of von Willebrand factor (vWF)
thyroxine replacement
Wilm's tumor
uncertain
tumor resection or chemotherapy
thrombocythemia
increased proteolysis of vWF
reduction in platelet count to normal
congenital heart valve defect
mechanical consumption of vWF
surgical repair of damaged valve
drugs
uncertain
drug discontinuation
Immune Cause
Mechanism
Therapy
SLE
antibody to vWF
corticosteroids
benign monoclonal gammopathy
antibody to vWF
does not respond to any conventional therapy including immunosuppression
The autoantibody in SLE and monoclonal gammopathy causes a deficiency in Factor 8 coagulant activity in addition to the deficiency in vWF.
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