Telangiectasia and von Willebrand's Disease in Two Families

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Abstract
Two families are described with members who have both von Willebrand''s disease and telangiectasias. Family A has 4 members in 3 consecutive generations that have both von Willebrand''s disease and telangiectasias. von Willebrand''s disease in this family is characterized by decreased ristocetin cofactor (FVIII-vWF), variably depressed factor VIII coagulant (FVIII-AHG), and factor VIII-related antigen (FVIII-AGN) levels. FVIII-AGN mobility on 2-dimensional crossed immunoelectrophoresis was normal. Four generations in Family B have von Willebrand''s disease characterized by decreased FVIII-AHG, FVIII-vWF, FVIII-AGN, and prolonged template bleeding times. Two members of this family also have telangiectasias and recurrent gastrointestinal bleeding. Results in these 2 families suggest an association between von Willebrand''s disease and telangiectasia.sbd.perhaps a defect in vascular endothelial cell function.