Pseudohemophilia Type B

On a Swedish Family with 51 Members Affected by von Willebrand's Disease

“…In middle age and later the pathologic tendency had practically disappeared. Similar observations have been made by other investigators (1,11,12,13,26,27).…”

In Memory of Erik Jorpes

“…In 1953 Alexander and Goldstein described 2 cases with a combined haemostatic defect, namely prolonged bleeding time and decreased factor VIII activity. Similar cases were later observed by several other workers (Larrieu & Soulier 1953, Quick & Hussey 1953, van Creveld et a1 1955, Singer & Ramot 1956, Schulman et a1 1956. This disorder was called vascular haemophilia, since most authors believed that the prolonged time was due to some defect in the vessel wall.…”

Plasma Antihaemophilic Factor (AHF) Concentrations in Families of Patients with Haemorrhagic States

“…The condition could be distinguished from haemophilia because of the existence of capillary abnormalities, the occurrence in females and the lack of evidence of a sex-hked inheritance. Further examples of a similar, if not identical disease, have been described by van Creveld, Jordan, Punt and Veder (1955), Darte (1955), Singer and Ramot (1956), Schulman, Smith, Erlandson, Fort and Lee (1956), Matter, Newcomb, Melly and Finch (1956), Fessey and Meynell (1957), Thomas, Black and Perry (1957), and Nilsson, Blomback and von Francken (1957). Some of these authors presented farmly studies to indicate that the deficiency of plasma AHF was inherited as an autosomal dominant.…”

“…Some of these authors presented farmly studies to indicate that the deficiency of plasma AHF was inherited as an autosomal dominant. On the other hand, the patient of Singer and Ramot (1956) was the offspring of a first cousin marriage, suggesting that in this instance a recessive gene was involved. The relationshp of h s condition to von Willebrand's disease has been clarified recently by Nilsson, Blomback, Jorpes, Blomback and Johansson (1957) who found plasma AHF deficiency to exist in the families originally investigated by von Wdebrand.…”