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Thromb Haemost 1981; 45(01): 073-076
DOI: 10.1055/s-0038-1650132
Original Article
Schattauer GmbH Stuttgart

Recent Investigations of the First Bleeder Family in Åland (Finland) Described by von Willebrand

D Nyman
1)   Department of Blood Coagulation Disorders, Karolinska Hospital, 104 01 Stockholm, Sweden
,
A W Eriksson
2)   Institute of Human Genetics, Free University, 1007 MC Amsterdam, The Netherlands
,
M Blombäck
1)   Department of Blood Coagulation Disorders, Karolinska Hospital, 104 01 Stockholm, Sweden
,
R R Frants
2)   Institute of Human Genetics, Free University, 1007 MC Amsterdam, The Netherlands
,
P Wahlberg
3)   Folkhälsan Institute of Genetics Unit, Helsiniki, Finland and Åland Central Hospital, Marieham, Åland, Finland
› Author Affiliations
Further Information

Publication History

Received 04 August 1980

Accepted 18 December 1980

Publication Date:
04 July 2018 (online)

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Summary

The still living members of the original bleeder family on the Åland Islands described by von Willebrand in 1926 have been reinvestigated by using modem laboratory techniques for the measurement of the Factor VIII complex and with regard to platelet aggregation. The low level of F VIII: C activity demonstrated in 1957 could be confirmed in some of the family members, who however all had only mild bleeding symptoms. More consistently, in 6 out of 10, a low F VIIIR: Ag was found; all of those also had a low F VIII: RCoF. In none of the members were excessively low values for any of the parameters found. However, the spectrum of the whole F VIII complex indicates that the original family described by von Willebrand belongs to von Willebrand’s disease, type I.

Keywords

von Willebrand’s disease - Åland Islands - Original family - F Vlll-complex - Platelet aggregation - Genetics
 

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