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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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