Augusta and Oscar ‘S’ were related and both had a history of nose bleeds, especially when they were younger; in both their families there had been ‘bleeders’. Augusta had already given birth to 11 children when Dr von Willebrand first saw her in 1924. By then, three daughters had died of bleeding complications (two from gastrointestinal bleeds at 2 years old and one at age 4 after a tongue bite). Hjördis, at that time aged 5 years, had among other bleeding problems been in bed for 10 weeks after a laceration to her lip and had also experienced a bad ankle bleed. She had a much prolonged Duke’s bleeding time

(according to Duke), while her coagulation time and clot retraction Selleckchem MK-2206 were normal. Her capillary fragility test was positive and platelet numbers were normal. von Willebrand ended his paper by stating the finding of a positive capillary resistance test does not necessarily mean that there is an alteration in the capillary vessel walls. He thought the pathogenesis of the bleeding was caused by platelet dysfunction, in combination with a general lesion of the vessel wall. Hjördis

bled to death at her fourth menstrual bleeding when she was 14 years old (Figs 3 and 4). When we first met the family in 1957, Åland was a poor country recovering from the Second World War. Professor Inga Marie Nilsson, from Malmö, was a guest researcher http://www.selleckchem.com/products/idasanutlin-rg-7388.html at Professor Erik Jorpe’s Medical Chemistry laboratory at Karolinska Institutet and I was employed at the laboratory. We had by then already investigated six Swedish families with pseudo-haemophilia and found that the probands and one parent, as well as several family members, had decreased levels of factor VIII, (FVIII) (at that time called AHG) and most had a prolonged bleeding time [2,3]. The probands had a

very prolonged bleeding time and an AHG level between 1% and 10% of normal. Infusion of human fraction I-0 (a purified fraction of Cohn Fraction I) corrected both the bleeding time and AHG deficiency and the capillary bleedings. We deduced by different in vivo experiments that there must be a new factor [4–7]. I thought the probands were homozygotes for the trait, but it was not until Zimmerman and co-workers developed a rabbit antibody to the FVIII/VWF Chlormezanone (FVIIIR:Ag) complex that real progress could be made. Many authors had by this time described similar patients with a severe haemorrhagic disorder (low AHG level and prolonged bleeding time) and had tried to find out if it was the same condition as that described by von Willebrand. Larrieu and Soulier suggested the name von Willebrand’s syndrome, which later became VWD. We, however, considered it distinguishable from von Willebrand’s thrombopathy or von Willebrand-Jurgens thrombopathy, as the German researchers preferred to call it [3]. However, Professor Jorpes came from Kökar (Fig. 1), another island in the Åland archipelago and he wanted us to investigate the Åland patients.