TY - CHAP M1 - Book, Section TI - Marginal Zone B-Cell Lymphomas A1 - Zinzani, Pier Luigi A1 - Broccoli, Alessandro A2 - Press, Oliver W. A2 - Lichtman, Marshall A. A2 - Leonard, John P. PY - 2017 T2 - Williams Hematology Malignant Lymphoid Diseases AB - SUMMARYIndolent B-cell lymphomas deriving from the marginal zone include three specific entities: extranodal marginal zone (or mucosa-associated lymphoid tissue) lymphoma (EMZL), splenic marginal zone lymphoma (SMZL), and nodal marginal zone lymphoma (NMZL). The clinical and molecular characteristics are distinctive for each of these entities, although some phenotypic and genetic features are overlapping. EMZL is the most common entity, arising at virtually any extranodal site, commonly associated with chronic antigenic stimulation either as a result of an external infection (e.g., Helicobacter pylori in the stomach) or an autoimmune disease (such as Sjögren syndrome or Hashimoto thyroiditis). SMZL accounts for approximately 20 percent of all marginal zone lymphomas, with patients typically presenting with an enlarged spleen and involvement of marrow and splenic hilar lymph nodes. NMZL is the least common entity, representing approximately 10 percent of all marginal zone lymphomas and typically presenting with lymph node–based disease without splenic or extranodal site involvement. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - hemonc.mhmedical.com/content.aspx?aid=1148368840 ER -