Emanuele Zucca

The term marginal zone lymphoma (MZL) is believed to be derived from B cells normally present in the marginal zone and was proposed in the Revised European-American Lymphoma (REAL) classification1 to take account of three apparently related lymphoma subtypes, namely the extranodal "low-grade B-cell lymphoma of mucosa-associated lymphoid tissue (MALT)" usually named MALT lymphoma, the nodal "monocytoid B-cell lymphoma," and the "primary splenic MZL with or without villous lymphocytes". At that time, the available cytogenetic data seemed to suggest that all three of these lymphomas share similar cytogenetic alterations, but several important cytogenetic and molecular genetic observations have later revealed the distinctiveness of these lymphoid neoplasms, and each is now considered a separate subtype in the World Health Organization (WHO) classification.2-4

While splenic and nodal MZL are very rare disorders, each comprising less than 1% of lymphomas,5 the extranodal MZL of MALT type is not uncommon. In a survey of more than 1400 non-Hodgkin's lymphomas from nine institutions in the United States of America, Canada, the United Kingdom, Switzerland, France, Germany, South Africa, and Hong Kong, this entity represented approximately 8% of the total number of cases, including both the most common gastrointestinal (GI) and the less usual non-GI localizations.5

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