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Splenic Marginal Zone Lymphoma

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Lymphoma

Part of the book series: Current Clinical Oncology ((CCO,volume 43))

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Abstract

Splenic marginal zone lymphoma (SMZL) shares a common cell of origin from the “marginal zone” (MZ), with extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) and nodal marginal zone lymphoma (NMZL). However, SMZL displays different clinical characteristics, reflecting probable biological variations according to the organ.

Within the past decade, new data regarding pathogenic mechanisms as well as therapeutic advances have been reported. Clinically, SMZL presents as an indolent disseminated disease at diagnosis with specific clinical presentation including predominant enlarged splenomegaly and autoimmune manifestations in 15 % of the patients. Diagnosis may be difficult among other small B-cell lymphomas, and the criteria for diagnosis have been recently improved. The therapeutic approaches comprise splenectomy or immunochemotherapy, but without consensus about the best treatment, except when associated with hepatitis C virus.

We are addressing here the current knowledge on the biological findings, clinical features, and therapeutic approaches for SMZL.

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Authors and Affiliations

  1. Department of Hemato-Oncologie, Hôpital Saint-Louis-APHP, 1 Av Claude Vellefaux, Paris, 75010, France

    Catherine Thieblemont MD, PhD

  2. Department of Hematology Biology, Hôpital Pitié–Salpetriere, APHP, 47-83 Boulevand de l’Hopital, Paris, 75013, France

    Frederic Davi PhD

  3. Department of Pathology, Hôpital Saint-Louis, APHP, 1 Av Claude Vellefaux, Paris, 75010, France

    Josette Brière MD, PhD

Authors
  1. Catherine Thieblemont MD, PhD
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  2. Frederic Davi PhD
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  3. Josette Brière MD, PhD
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Correspondence to Catherine Thieblemont MD, PhD .

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Editors and Affiliations

  1. Holcombe Blvd. 1515, Houston, 77030, Texas, USA

    Anas Younes

  2. Service d'Hematologie, CHU Lyon-Sud, chemin du Grand Revoyet, Pierre Bénite, 69310, France

    Bertrand Coiffier

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Thieblemont, C., Davi, F., Brière, J. (2013). Splenic Marginal Zone Lymphoma. In: Younes, A., Coiffier, B. (eds) Lymphoma. Current Clinical Oncology, vol 43. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-62703-408-1_7

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  • DOI: https://doi.org/10.1007/978-1-62703-408-1_7

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