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GE Marti, ME Rick, J Sidbury and HR Gralnick
Five patients with glycogen storage disease type I (GSD-I) were evaluated
for a bleeding diathesis and subsequently were given an infusion of
1-deamino-8-D-arginine vasopressin (DDAVP). Although platelet counts were
normal or slightly elevated, the baseline template bleeding times were
prolonged in four of the patients. Prothrombin times and activated partial
thromboplastin times were normal, while ADP- and epinephrine-induced
platelet aggregations were absent in the three patients tested. Ristocetin-
and collagen-induced platelet aggregations were abnormal. Laurell and
immunoradiometric determinations of the factor VIII-related antigen (vWf
antigen) were decreased. Glyoxyl agarose gel electrophoresis of the
patients' plasma revealed abnormal multimer patterns in four of the five
patients. After the DDAVP infusion the platelet aggregation abnormalities
persisted; however, the bleeding time and the von Willebrand antigen and
activity corrected. We conclude that GSD-Ia patients may have a
metabolically acquired form of von Willebrand's syndrome as well as an
acquired intrinsic platelet defect, and that DDAVP may be useful in the
management of bleeding in these patients.
This article has been cited by other articles:
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| Copyright © 1986 by American Society of Hematology Online ISSN: 1528-0020 | |||||||||
