Acquired von Willebrand syndrome in systemic lupus erythematodes.
Acquired von Willebrand syndrome (AvWS) in systemic lupus erythematodes (SLE) is caused by autoantibodies directed against the circulating von Willebrand factor (vWF)/factor VIII (FVIII) complex. The autoantibody-vWF/FVIII antigen complex is cleared rapidly from the circulation, leading to a moderate to severe quantitative and qualitative deficiency of both vWF and FVIIIc. Consequently, AvWS in SLE is featured by a prolonged bleeding time and normal platelet count, a prolonged activated partial thromboplastin time (APTT) and normal prothrombin time (PT), decreased or absent ristocetin-induced platelet aggregation (RIPA), and type II vWF deficiency on multimeric analysis of the vWF protein. Acquired von Willebrand syndrome type II in SLE responds poorly to 1-desamino-8-D-arginine vasopressin (DDAVP) and FVIII concentrate, but responds transiently well to high-dose gammaglobulin given intravenously. All reported cases of AvWS in SLE were cured by appropriate treatment of the underlying autoimmune disease with prednisone or immunosuppression.
Michiels
J J
JJ
Clinical Hemostasis and Thrombosis, Department of Hematology, University Hospital Antwerp, Edegem, Belgium. postbus@goodheartcenter.demon.nl
Schroyens
W
W
van der Planken
M
M
Berneman
Z
Z
D000293:Adolescent; D000328:Adult; D000368:Aged; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D003894:Deamino Arginine Vasopressin / Q000008:administration & dosage; D005169:Factor VIII / Q000008:administration & dosage; D005260:Female; D006470:Hemorrhage; D006490:Hemostatics / Q000008:administration & dosage; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000097:blood / Q000150:complications*; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000008:administration & dosage; D012720:Severity of Illness Index; D016896:Treatment Outcome; D014842:von Willebrand Diseases / Q000188:drug therapy* / Q000209:etiology*
D000893:Anti-Inflammatory Agents; D006490:Hemostatics; D005169:Factor VIII; D003894:Deamino Arginine Vasopressin; D011241:Prednisone