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Type IIB von Willebrand's Disease: Unusual Response to Cryoprecipitate Infusion

RONALD S. WEINGER, MD.; PHILIP L. CIMO, M.D.; JOEL L. MOAKE, M.D.; JOHN D. OLSON, M.D., Ph.D.; and MICHAEL S. HELLER, M.D.
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Grant support: in part by grant MC-B-480001-01-0 from the U. S. Department of Health and Human Resources.

▸Requests for reprints should be addressed to Ronald S. Weinger, M.D.; Division of Hematology, Department of Medicine, Boston Veterans Administration Medical Center, 150 South Huntington Avenue; Boston, MA 02130.


Houston, Texas


Ann Intern Med. 1981;94(1):47-50. doi:10.7326/0003-4819-94-1-47
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A 16-year-old boy had IIB von Willebrand's disease. The disorder is characterized by prolonged bleeding times; normal plasma levels of factor VIII-coagulant activity, factor VIII-ristocetin cofactor activity, and factor VIII-related antigen; abnormal (anodal) mobility of plasma factor VIII-related antigen on two-dimensional crossed Immunoelectrophoresis; and enhanced binding of plasma factor VIII-related antigen to normal platelets in the presence of ristocetin. These variables were measured at time periods after an infusion of normal cryoprecipitate into the patient. The electrophoretic mobility of his plasma factor VIII-related antigen was normal 15 minutes after the infusion but became abnormal (anodal) by 4 hours. His bleeding times were normal after 24 hours and did not correlate with plasma levels of factor VIII-coagulant activity, factor VIII-ristocetin cofactor, factor VIII-related antigen, or the electrophoretic mobility of his plasma factor VIII-related antigen. These results imply that the abnormal factor VIII/von Willebrand factor multimers in the plasma of these patients can associate with normal factor VIII/von Willebrand factor multimers and delay the deposition of the normal multimers into subendothelial surfaces. This may require cryoprecipitate infusions 24 hours before elective surgical procedures.

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