|
Diagnosis and treatment of von Willebrand disease: new perspectives and nuances
In 1926, Erik von Willebrand, a Finnish internist and
academic, evaluated and described a 5-year-old girl
with extreme bleeding and bruising due to what had
been designated the A ° landic haemorrhagic disease
by inhabitants of this archipelago island in the Gulf
of Bothnia. Hjørdis, the propositus, was the ninth of
12 children born to a pedigree in which four female
siblings had already died before the age of 4 with
uncontrolled haemorrhage and in which 23 of 66
family members, predominantly females, had experienced
significant bleeding and bruising complications
[1]. In fact, Hjørdis herself eventually died at
the age of 13 during her fourth menstrual cycle.
Professor von Willebrand mistakenly concluded that
this bleeding diathesis was an unusual form of
haemophilia and decided to label the new disease
as pseudo-haemophilia to differentiate it from the
sex-linked recessive haemophilia A.
点击下载全文：血管性血友病的诊断治疗最新进展
|
