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#1 10-12-2006 17:32:52

destivic
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Lieu: Nantes
Date d'inscription: 30-11-2006
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Hémophilie

L'hémophilie est une anomalie constitutionnelle de la coagulation sanguine en rapport avec un déficit d’un des facteurs de la coagulation. Les manifestations cliniques de la maladie sont proportionnelles au déficit du facteur de la coagulation. Les deux manifestations cliniques les plus caractéristiques de la maladie sont les hémorragies dans les articulations (hémarthroses) et dans les muscles (hématomes).
La maladie peut être sévère avec manifestations dès la première année de vie ou légère avec très peu de manifestations.
Il existe deux types d’hémophilie en rapport avec des déficits de facteurs de coagulation différents :

    - L'atteinte du gène du facteur VIII correspond à l’hémophilie A
    - L'atteinte du gène du facteur IX à l’hémophilie B



Description

Le sang d'une personne atteinte d'hémophilie ne coagule pas normalement. Les saignements ne sont pas plus abondants, ni plus rapides que la normale, mais durent plus longtemps. Bien des gens croient que les hémophiles saignent beaucoup à la moindre petite coupure. c'est un mythe. Les blessures superficielles ne sont en général pas graves, elles ne saignent pas plus que chez un non hémophile sinon un simple sparadrap permet d'arrêter le saignement.
Les saignements internes, par contre, ont de beaucoup plus graves conséquences, notamment s'ils concernent les articulations, surtout les genoux, les chevilles et les coudes, ainsi que les tissus mous et les muscles.



Sévérité

La gravité de l'hémophilie est fonction du pourcentage d’activité du facteur de coagulation déficitaire. L'activité normale est située entre 50 et 150 %, correspond à l’intervalle normal d’activité du facteur de coagulation VIII ou IX dans le sang.


    - L'hémophilie légère : entre 5 et 30 % de l’intervalle normal d’activité du facteur de coagulation.
           Symptômes :
                - Peut ne jamais présenter de trouble de saignement
                - Pas de saignement à moins d’une blessure
                - Risque de saignement prolongé après une intervention chirurgicale ou une lésion grave
                - Hémorragies rares.


    - L'hémophilie moyenne : entre 1 et 5 % de l’intervalle normal d’activité du facteur de coagulation.
           Symptômes :
                - Risque de saignement prolongé après une intervention chirurgicale, une lésion grave ou une intervention dentaire.
                - Peut présenter des épisodes de saignement environ une fois par mois.
                - Saigne rarement, voire jamais sans raison évidente.


    - L'hémophilie grave : moins de 1 % de l’intervalle normal d’activité du facteur de coagulation.
           Symptômes :
                - Hémorragies fréquentes au niveau des muscles ou des articulations (principalement les genoux, les coudes et les chevilles).
                - Peut présenter des épisodes de saignement une ou deux fois par semaine.
                - Peut saigner sans raison évidente.


Source : encyclopédie wikipedia


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