Eight Factor

What is Hemophilia?

Type A haemophilia is an inherited genetic disease that involves the partial or total reduction of the activity of a specific protein: Factor VIII.

This anomaly causes an alteration in the blood coagulation process, which exposes those affected to a greater risk of bleeding episodes both internal and external, potentially very dangerous if not properly treated.

Today, extremely effective therapies are available on the market, such as replacement therapy, which makes it possible to integrate the missing Factor VIII, thus allowing optimal management of the disease.

Thanks to these treatments, subjects suffering from haemophilia can thus lead an almost normal life and rely on a life expectancy comparable to that of a healthy subject.

8 THINGS TO KNOW FOR A BETTER LIFE WITH HAEMOPHILIA

Wikiphilia

Learn more about the meaning of the words you read on this page and learn about the entire glossary on hemophilia.

Phase of haemostasis during which the clot forms.

It is a complex process constituted by a number of biochemical reactions that follow one another according to a specific sequence and that involve a number of proteins, including clotting factors, thrombin and fibrinogen.

A solid mass constituted by a clump of platelets held together by a mesh constituted by a protein called fibrin.

The function of the clot is to plug the opening that forms in the wall of a damaged blood vessel, in order to prevent blood loss.

Once the damage has been repaired, the clot is dissolved by specific mechanisms triggered by our body.

A group of proteins with enzymatic functions involved in the clotting process.

All clotting factors are assigned a Roman numeral, although each one also has a proper name.

Some are produced by the liver, whereas others are synthesised by the endothelial cells that make up the inner lining of the blood vessels.

Fuoriuscita di sangue dai vasi sanguigni che si verifica in seguito al danneggiamento delle loro pareti.

L’emorragia si definisce interna nei casi in cui il sangue defluisce in una cavità naturale del corpo, o esterna quando il sangue si riversa all’esterno o in una cavità comunicante con l’esterno.

A protein belonging to the clotting factor category, a group of enzymes involved in the blood-clotting process.

It is encoded by a gene on the long arm of the X chromosome.

Factor VIII is also known as anti-haemophilic factor (AHF).

Product containing a high concentration of freeze-dried factor VIII, used for replacement therapy in the treatment of haemophilia A.

A bleed that occurs in a joint, i.e. the point at which two bones meet.

Haemarthroses are the most common bleeding events in patients with haemophilia and cause the swelling and redness of the joint, which becomes warm and painful.

The joint’s prolonged exposure to the inflammation generated by the build-up of blood can lead to the development of joint disease.

The production and maturation of the corpuscular blood cells, i.e. the red blood cells, white blood cells and platelets.

After birth, haematopoiesis occurs predominantly in the bone marrow and in the lymphatic organs.

A ereditary genetic illness, characterised by a deficiency of clotting factor VIII, that exposes the individual to a greater risk of both internal and external bleeds.

Haemophilia A is more common in males, whereas females tend to be healthy carriers of the condition.

The typical symptoms of the condition include haemarthroses (joint bleeds) and haematomas (muscle bleeds).

Antibodies produced by the immune system of patients with haemophilia, following an infusion of replacement factor VIII.

The inhibitors recognise the infused factor VIII and bind with it in a specific manner, thereby neutralising its effect.

Damage that occurs in the joints and prevents their normal function, thereby impairing the individual’s ability to move normally. In haemophiliac patients, joint disease is usually the consequence of recurrent haemarthroses.

A substance extracted from plasma, and subjected to a thorough process of filtration, purification, viral inactivation and control, in order to confirm its purity, and the absence of contaminants.