Blood consists of a liquid called plasma, red blood cells,
white blood cells and platelets. Red bloods cells deliver oxygen from your
lungs to your tissues and organs, white blood cells fight infection as part of
your body's defense system, and platelets help blood clot when you experience a
cut or wound. Blood is a constantly circulating fluid providing the body with
nutrition, oxygen, and waste removal. Your blood group depends on which
antigens occur on the surface of your red blood cells.
What is Hemophilia? What is
Haemophilia?
Hemophilia is a group of inherited blood disorders in which the
blood does not clot properly. Hemophilia is the standard international
spelling, also known as haemophilia in the UK, other translations include:
hémophilie, hemofilie, hemofili, hemofilia, hämophilie, emofilia. We will use
the standard international spelling for the purpose of this section.
Bleeding
disorders are due to defects in the blood vessels, the coagulation mechanism,
or the blood platelets. An affected individual may bleed spontaneously or for
longer than a healthy person after injury or surgery.
The blood
coagulation mechanism is a process which transforms the blood from a liquid
into a solid, and involves several different clotting factors. The mechanism
generates fibrin when it is activated, which together with the platelet plug,
stops the bleeding.
When coagulation
factors are missing or deficient the blood does not clot properly and bleeding
continues.
Patients with
Hemophilia A or B have a genetic defect which results in a deficiency in one of
the blood clotting factors.
Queen Victoria
was a carrier and passed the mutation to her son Leopold, and through several
of her daughters to members of the royal families of Spain, Russia, and
Germany.
Tsarevich Alexei
Nikolaevich, son of Nicholas II (Russia) suffered from hemophilia and was a
descendant of Queen Victoria - Rasputin was successful in treating his
hemophilia, it was claimed.
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