Hemophilia a and Variant of von Willebrand’s Disease in one Family

A 6 years old boy with the admitting diagnosis hemophilia A was transferred to our hospital because of persistent bleeding episodes inspite of adequate treatment with AHF. Laboratory data were as follow: F VIII coagulant activity 1.7%, inconstantly prolonged bleeding time (hemorrhagometry), inconstantly reduced platelet adhesiveness, normal IVY-bleeding time, normal platelet aggregation with ADP and collagen, reduced Ristocetin-induced aggregation with PRP, reduced Ristocetin-cofactor activity, normal F VIII associated antigen. An inhibitor against F VIII could be excluded. The bleeding stopped and the pathological coagulation findings were corrected after substitution therapy with isoagglutininfree cryoprecipitate. Because of these findings we made the diagnosis of variant of von Willebrand’s disease. His 8 years old brother had classical hemophilia A with a F VIII coagulant activity of 1.8% and all other coagulation parameters mentioned above within normal limits. In the mother a carrier state could not be detected. In the mothers and fathers family bleeders are not known.

Angaben zum Objekt

• Standort: Deutsche Nationalbibliothek Frankfurt am Main
• Umfang: Online-Ressource
• Sprache: Englisch

Verweise und Beziehungen

• Erschienen in: Hemophilia a and Variant of von Willebrand’s Disease in one Family ; volume:38 ; number:05 ; year:1977 ; Konferenz: XIIth Congress of The World Federation of Hemophilia; 1977-06-22; Philadelphia ; Thrombosis and haemostasis ; 38, Heft 05 (1977)

Beteiligte, Orts- und Zeitangaben

• Beteiligte Personen und Organisationen: Sutor, A.H. ; Böttcher, D. ; Hasler, K.

Weitere Informationen

• DOI: 10.1055/s-0039-1682607
• URN: urn:nbn:de:101:1-2412190856432.194779058692
• Rechteinformation: Open Access unbekannt; Der Zugriff auf das Objekt ist unbeschränkt möglich.
• Letzte Aktualisierung: 15.08.2025, 07:23 MESZ