VASCULAR HEMOPHILIA

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Abstract
The coagulation and vascular aspects of the von Willebrand syndrome have been studied in seven children. Six of the children demonstrated a severe defect in the coagulation mechanism which was found to result from a marked deficiency in antihemophilic globulin. The latter deficiency was found in boys and girls, was familial, and apparently occurred in a manner distinct from the sex-linked recessive inheritance of classical hemophilia. Morphologic capillary abnormalities were noted in the nail-beds of the fingers and in the bulbar conjunctivae of children with and without associated deficiency of antihemophilic globulin. Administration of fresh plasma resulted in correction of both bleeding time and coagulation defects in a patient with the combined vascular-coagulation abnormality. The vascular abnormality was found to be independent of deficiency of antihemophilic globulin or any of the known platelet factors. Classification of the von Willebrand syndrome into at least two groups was recommended: patients with vascular defect plus AHG deficiency (vascular hemophilia), and patients with vascular defect and normal coagulation status (pseudohemophilia).

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