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#1 13-12-2006 00:03:53

destivic
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Lieu: Nantes
Date d'inscription: 30-11-2006
Messages: 140
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Maladie de Vaquez

La maladie de Vaquez est une hémopathie myéloproliférative caractérisée par une polyglobulie et une augmentation du volume globulaire total.



Physiopathologie


Proliferation myéloïde clonale prédominant sur la lignée érythrocytaire au niveau medullaire et splénique.



Diagnostic



Signes cliniques

    - maladie de l'adulte après 50 ans
    - Hypertension artérielle
    - Prurit et érythrose cutanée
    - Splénomégalie
    - Thromboses veineuses et artérielles et hémorragie
    - Ulcère gastrique
    - Priapisme (cet élément clinique me paraît peu établi)



Signes biologiques

    - hémogramme :
          - augmentation de l'hématocrite
          - augmentation du taux d'hémoglobine
          - augmentation du nombre de globules rouges
          - hyperleucocytose et myélémie
          - thrombocytose

    - Volume globulaire total augmenté
            > 35 ml/kg chez l'homme
            > 32 ml/kg chez la femme



Complications

    - thromboses et hémorragies par thrombopathie et polyglobulie (risque au-delà de 55% d'hématocrite)
    - hyperuricémie (goutte, coliques néphrétiques)
    - insuffisance mlédullaire par myélofibrose après 10 ans d'évolution
    - acutisation en leucémie aïgue dans 10 % des cas



Traitement

    - Pronostic : médiane de survie à 10 ans, risque d'insuffisance médullaire, de leucémie aïgue

    - Traitement symptomatique :
          - thrombocytose : aspirine à dose antiagrégante (75 à 160 mg/j)
          - hyperuricémie : allopurinol (Zyloric) et hydratation alcaline (vichy 2l/j)
          - prurit : antihistaminique H1 type cétirizine (Zyrtec)

    - Traitement spécifique :
          - Saignées
            pour des sujets jeunes ou lors de grossesse ou en urgence si l'hématocrite >55 %
               contre-indiqué si les plaquettes > 800 000 sauf en urgence
               entraîne une carence martiale à respecter
               traitement contraignant et risque de thrombocytoses secondaires
            200 à 300 ml avec ou non réinjection du plasma
               2 à 3 fois par semaine en urgence, puis tous les 1 à 3 mois
   
          - Hydroxyurée (Hydrea) ou Pipobroman (Vercyte)

              sujet non compliants aux saignées ou en cas de thrombocytose
              peut être associé aux saignées

          - Phosphore 32
              sujet âgé de plus de 75 ans ou avec une espérance de vie limitée (de plus en plus abandonné)
                  risque accru de leucémie aigüe
            injection IV unique, suivie d'une deuxième injection à 2 mois en cas d'échec
                  effet retardé de 2 mois, contrôle la maladie pendant plusieurs années
                  renouvelable en cas de récidive

          - Interféron
                  place non définie

          - Autre
                 suveillance hémogramme tous les 2 mois minimum
                 prise en charge à 100 %




Source : encyclopédie wikipedia


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