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Splenic Marginal Zone Lymphoma

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Indolent Lymphomas

Part of the book series: Hematologic Malignancies ((HEMATOLOGIC))

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Abstract

Splenic marginal zone lymphoma (SMZL) is a rare B-cell neoplasm showing a preferential homing in spleen and bone marrow. The spread of neoplastic cells to the lymphatic organs takes the appearance of a micronodular infiltration. A reliable diagnosis of SMZL can be formulated relying on bone marrow histology, particularly if pathological findings are reasonably integrated with peripheral blood morphology/phenotype data and detailed clinical information. However, due to lack of a distinct cellular marker, without the support of spleen histology or biomolecular and cytogenetic investigations, the differential diagnosis with other indolent B-cell neoplasia may prove to be challenging. SMZL is an indolent neoplasm with median survival around 10–15 years, yet the clinical course is quite heterogeneous, and about 20% of patients experience a more aggressive clinical pace and shorter survival. An evolution towards more aggressive histology, mostly diffuse large B-cell lymphoma (DLBCL) occurs in around 10% of cases. Since prospective phase III trials are lacking, the treatment is not standardised. Currently, the aim of treatment is primarily palliative, and outside of a clinical study, it is wise and clinically sound to rely on therapies associated with a low risk of morbidity and mortality. Both rituximab as monotherapy and splenectomy comply with these characteristics and produce a high rate of sustained clinical response; thus, either rituximab or splenectomy is presently the most reasonable therapeutic choice.

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Authors and Affiliations

  1. Haematology and BMT Unit, Department of Oncology, Casa di Cura La Maddalena, Palermo, Italy

    Emilio Iannitto

  2. APHP, Saint-Louis Hospital, Hemato-Oncology – Paris Diderot University, Paris, France

    Catherine Thieblemont

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  1. Emilio Iannitto
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  1. Department of Medicine III, LMU Hospital, München, Bayern, Germany

    Martin Dreyling

  2. Azienda Ospedaliera Nazionale SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy

    Marco Ladetto

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Iannitto, E., Thieblemont, C. (2021). Splenic Marginal Zone Lymphoma. In: Dreyling, M., Ladetto, M. (eds) Indolent Lymphomas . Hematologic Malignancies. Springer, Cham. https://doi.org/10.1007/978-3-030-55989-2_9

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