Acquired von Willebrand's syndrome with lupus-like serology.
We describe a 12-year-old boy with acquired von Willebrand's syndrome, who also had various autoantibodies. He presented with recent hemorrhagic symptoms and a prolonged bleeding time. Hemostatic studies revealed severely reduced levels of factor VIII procoagulant activity (VIII:C), von Willebrand's factor (vWF) antigen (vWF:Ag), and ristocetin cofactor activity (RCoF). An inhibitor that could be detected in the patient's plasma moderately decreased the levels of vWF:Ag in normal plasma, but did not interfere with the measurement of VIII:C or RCoF. Following the infusion of cryoprecipitate, half-lives of VIII:C, vWF:Ag, and RCoF were markedly reduced. 1-Deamino-8-D-arginine vasopressin infusion induced normalization of the prolonged bleeding time and caused a marked increase in VIII:C, vWF:Ag, and RCoF. Prior to treatment, there was a uniform reduction of all the multimers of plasma vWF in sodium dodecyl sulfate agarose gel electrophoresis. Following prednisone therapy, clinical and hemostatic findings were improved, and the multimeric patterns of vWF were normalized. These findings suggest that the low levels of all three parameters of factor VIII and all the multimers of plasma vWF in the patient are caused by rapid elimination of factor VIII complex from the circulation.
Igarashi
N
N
Department of Pediatrics, Kanazawa University School of Medicine, Japan.
Miura
M
M
Kato
E
E
Matsuda
T
T
Itoh
K
K
Abildgaard
C F
CF
Shimizu
M
M
Koizumi
S
S
Sato
T
T
Taniguchi
N
N
D002648:Child; D004587:Electrophoresis, Agar Gel; D005169:Factor VIII / Q000037:antagonists & inhibitors; D005500:Follow-Up Studies; D006801:Humans; D008177:Lupus Vulgaris / Q000097:blood / Q000276:immunology*; D008297:Male; D011241:Prednisone / Q000627:therapeutic use; D014842:von Willebrand Diseases / Q000097:blood / Q000276:immunology*
D005169:Factor VIII; D011241:Prednisone