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Mechanisms of Lymphomagenesis in Celiac Disease: Lessons for Therapy

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Refractory Celiac Disease

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Abstract

After summarizing historical studies which established lymphomas as one rare but most severe complication of celiac disease and demonstrated their origin from intraepithelial lymphocytes, we discuss how recent work has allowed to identify their precursor within an unusual subset of innate-like lymphocytes, to define their complex mutational landscape dominated by driver mutations in the JAK1-STAT3 pathway and thereby to propose a scenario through which chronic inflammation promotes lymphoma development with or without the intermediary step of intraepithelial lymphoma that defines type 2 refractory CeD (RCD2). We next examine how this pathogenic scheme might guide therapeutic approaches to prevent progression from RCD2 into overt high-grade enteropathy-associated lymphomas, and improve long term prognosis.

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Authors and Affiliations

  1. Université de Paris, Imagine Institute, Laboratory of Intestinal Immunity, INSERM UMR1163, Paris, France

    Sascha Cording & Sofia Berrabah

  2. Laboratory of Onco-Haematology, AP-HP, Hôpital Necker Enfants-Malades, Paris, France

    Ludovic Lhermitte

  3. Department of Gastroenterology, AP-HP. Centre-Université de Paris Hôpital Cochin, Paris, France

    Georgia Malamut

  4. Université de Paris, Imagine Institute, Laboratory of Intestinal Immunity, INSERM UMR1163, Paris, France

    Georgia Malamut

  5. Laboratory of Intestinal Immunity, Université de Paris, INSERM UMR 1163 and Imagine Institute, Paris, France

    Nadine Cerf-Bensussan

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  1. Sascha Cording
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  2. Sofia Berrabah
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  3. Ludovic Lhermitte
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  4. Georgia Malamut
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  5. Nadine Cerf-Bensussan
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Correspondence to Nadine Cerf-Bensussan .

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Editors and Affiliations

  1. Department of Gastroenterology, AP-HP. Centre-Université de Paris Hôpital Cochin, Paris, France

    Georgia Malamut

  2. Laboratory of Intestinal Immunity, Université de Paris, INSERM UMR 1163 and Imagine Institute, Paris, France

    Nadine Cerf-Bensussan

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Cording, S., Berrabah, S., Lhermitte, L., Malamut, G., Cerf-Bensussan, N. (2022). Mechanisms of Lymphomagenesis in Celiac Disease: Lessons for Therapy. In: Malamut, G., Cerf-Bensussan, N. (eds) Refractory Celiac Disease. Springer, Cham. https://doi.org/10.1007/978-3-030-90142-4_3

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