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La maladie de Waldenström est une rare maladie hématologique, caractérisée par une prolifération lymphocytaire monoclonale B avec infiltration des organes lymphoïdes périphériques et de la moelle osseuse par une population lymphoplasmocytaire monoclonale sécrétant une IgM monoclonale. Elle est d'étiologie inconnue mais une prédisposition génétique familiale semble établie. Elle touche les hommes plus que les femmes (sex ratio de 3 pour 1). L'âge de prédilection est entre 50 et 70 ans.
Tableau clinique
La maladie de Waldenström est le plus souvent asymptomatique et découverte fortuitement devant une VS très élevée (> 70 voire 100 mm). On retrouve parfois les signes cliniques suivants :
- Asthénie progressive ;
- Adénopathies dans 20 à 30% des cas, fermes, indolores, non adhérentes et le plus souvent cervicales, axillaires ou inguino-crurales ;
- Splénomégalie ou hépatosplénomégalie modérée ;
- Syndrome hémorragique cutanés, muqueux voire rétiniens ;
- Syndrome d'hyperviscosité ;
- Neuropathie périphérique dans 5 à 10% des cas, symétrique, sensitive.
Biologie
La Numération Formule sanguine est souvent normale. On retrouve parfois certaines anomalies non spécifiques :
- Anémie arégénérative modérée à sevère, normocytaire ou faussement macrocytaire, hématies en rouleaux ;
- Hyperleucocytose modérée (< 15 000) ou neutropénie modérée, monocytose modérée, lyphocytose normale ou augmentation modérée ;
- Plaquettes normales ou thrombopénie dans 10% des cas ;
- Allongement du temps de saignement par thrombopathie ;
- VS très accélérée (> 70 mm voir 100 mm à la 1ère heure) ;
- Hyperprotidémie > 80 voir 120 g/l ;
- L'électrophorèse des protéines met en évidence un pic monoclonal béta ou gamma ;
- L'immunoélectrophorèse mettent en évidence une IgM monoclonale k dans 75% des cas ou l dans 25% des cas, avec IgG et IgA normales ou abaissées ;
- Calcémie normale ;
- Protéinurie de Bence-Jones : chaînes légères libres dans les urines ;
- Protéinurie totale faible.
Myélogramme
Il met en évidence une : une infiltration lymphoïde polymorphe représentant de 20 à 100 % des cellules medullaire
- Infiltration lympho-plasmocytaire polymorphe de la moelle ;
- Fibrose et mastocytose réactionnelle, inconstantes ;
Diagnostic différentiel
- Le myélome à IgM (2% des myélomes) : insuffisance rénale, protéinurie, hypercalcémie plus fréquentes, évolution plus rapide ;
- Le syndrome de Schnitzler : urticaire chronique, ostéocondensation et IgM monoclonale ;
- Les rares leucémies lymphoïdes chroniques avec pic d’IgM < 5 g/L ;
- Les gammapathies monoclonales bénignes ou "de nature indéterminée" (MGUS monoclonal gammapathy of undetermined signifiance) à IgM : le taux est souvent plus faible, pas d'infiltration médullaire, stabilité sur 3 ans ;
- Les lymphomes non sécrétants ;
- Des IgM monoclonales peuvent être retrouvées lors d'infections, de carcinomes, de maladies auto-immunes, de lymphomes B sécrétants.
Traitements
L'évolution spontanée est variable et dans certains cas la maladie reste stable pendant des années (2 à 10 ans).
1. Dans les formes asymtomatiques avec un taux stable de l'IgM, on propose l'absention thérapeutique seule avec surveillance.
2. Dans les formes symptomatiques et/ou avec une augmentation des taux de l'IgM, on propose différents types de chimiothérapie, en fonction du stade tumoral :
- Dans les formes lente, on propose une mono chimiothérapie orale continue ;
- Dans les formes sévères rapide et/ou compliquées, on propose une polychimiothérapie ;
- Le sujet agé est traité par monochimiothérapie ;
- En cas de manifestation auto-immunes on associe la prednisone avec le chlorambucil, le cyclophosphamide et le melphalan ;
1. Traitement symptomatique des complications et notamment de l'anémie ;
2. En cas de syndrome d'hyperviscosité ou de neuropathie, on propose des plasmaphérèses.
Source : encyclopédie wikipedia
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