Résumé
Les lymphomes dérivant de la zone marginale (LZM) regroupent au moins trois entités se distinguant par le site anatomique où siège la prolifération tumorale: les lymphomes extraganglionnaires développés à partir du tissu lymphoïde associé aux muqueuses (MALT), les lymphomes spléniques et les lymphomes ganglionnaires. Les LZM représentent environ 10 % de l’ensemble des lymphomes malins, et sont décrits comme pouvant être associés à une stimulation antigénique chronique représentée par des pathogènes microbiens ou par des autoantigènes. Leur présentation est hétérogène car elle dépend du site d’envahissement, mais elle a en commun l’absence de facteur de mauvais pronostic au diagnostic, l’absence de symptômes B, et une évolution clinique indolente. Le diagnostic différentiel est représenté par le lymphome lymphoplasmocytaire ou maladie de Waldenström, décrit comme dérivant aussi d’une cellule de la zone marginale. En raison de la faible incidence des LZM et de leur reconnaissance histologique parfois difficile, on ne dispose pas de conduite thérapeutique standardisée.
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Thieblemont, C., Davi, F., Brière, J. (2013). Lymphomes de la zone marginale. In: Actualités thérapeutiques dans les lymphomes. Springer, Paris. https://doi.org/10.1007/978-2-8178-0371-5_8
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DOI: https://doi.org/10.1007/978-2-8178-0371-5_8
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