NCI Definition: A B-cell non-Hodgkin lymphoma composed of small lymphocytes which surround and replace the splenic white pulp germinal centers. It involves the spleen and splenic hilar lymph nodes, bone marrow, and often the peripheral blood. When lymphoma cells are present in the peripheral blood, they are usually, but not always, characterized by the presence of short polar villi. (WHO) [1]

Significant Genes in Splenic Marginal Zone Lymphoma


BCL2 +

BCL6 +


IRF4 +

JAK2 +

JAK3 +


MYD88 +

TYK2 +

Disease Details

Splenic Marginal Zone B-Cell Lymphoma, Marginal Zone Lymphoma of the Spleen, Marginal Zone Lymphoma of Spleen, SLVL, Splenic Marginal Zone B-Cell Lymphoma with Villous Lymphocytes, Splenic Lymphoma with Circulating Villous Lymphocytes, SMZL, Splenic Marginal Zone Lymphoma with Villous Lymphocytes
Marginal Zone Lymphoma
OncoTree Name
Splenic Marginal Zone Lymphoma
OncoTree Code


1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 6. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.