Historia de la Hemofilia

Se publica en The Lancet el primer tratamiento médico con éxito de la hemofilia; un niño de once años que llevaba seis días sangrando después de una intervención quirúrgica se recuperó gracias a una transfusión de sangre completa.

An American vet observes a large number of deaths through bleeding in cattle that had eaten mouldy sweet clover. The doctor Karl LINK and collaborators identify the molecule responsible in this clover: dicoumarol, the antidote for which is vitamin K.

König describes for the first time relationship between haemophilia and joint disease. Today 70-80% of blee- ding in haemophiliac patients occur in the joints and arthropathy is the most important long term complication.

Patek and Taylor (Boston) find that the coagulation defect of haemophiliac patients could be corrected by adding a substance extracted from plasma, which they call "anti-haemophilic globulin". This substance is actually known as Factor VIII.

Distinction by Rosemary Biggs (Oxford) of a disease entity different from classic haemophilia, which has been called "Christmas disease", after the last name of the first child described with the condition. It is now called haemophilia B, while "classic" haemophilia is called haemophilia A.

Judith G Pool (Stanford) finds that plasma cryoprecipitate contains a large amount of factor VIII and could be used to efficiently tread haemophiliac patients. This is an enormous step forward in haemophiliac patients’ care, forsaking the use of whole blood and fresh plasma.

First Haemophiliac patient with HIV infection is reported. Most of non-virus-inactivated clotting factor concentrates, made from thousands donors, were unknowingly contaminated by blood-borne viruses, HIV and hepatitis viruses. The "golden era" of haemophilia management is prematurely ended.

Development and implementation of safe virus inactivated plasma-derived factors. This represents a major breakthrough ending the black period of blood borne viruses contamination.

First report of the efficiency of pathogen-free recombinant FVIII therapy in two patients with Haemophilia A. The molecule become available for haemophilia A patients in the same year.

While treatments of haemophilia become safer, knowledge about the

development of inhibitor against factor concentrates greatly increases. Inhibitors are now the major drawback of exogenous factor replacement therapy.exploration path of hemostasis.

Prolonged half-life factor concentrates are actually being implemented in clinical practice to improve patient's compliance and quality of life.