TY - CHAP M1 - Book, Section TI - Marginal Zone B-Cell Lymphomas A1 - Broccoli, Alessandro A1 - Zinzani, Pier Luigi A2 - Kaushansky, Kenneth A2 - Prchal, Josef T. A2 - Burns, Linda J. A2 - Lichtman, Marshall A. A2 - Levi, Marcel A2 - Linch, David C. PY - 2021 T2 - Williams Hematology, 10e 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 overlap. 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 (eg, Helicobacter pylori in the stomach) or an autoimmune disease (eg, Sjögren syndrome or Hashimoto thyroiditis). SMZL accounts for approximately 20% of all marginal zone lymphomas (MZLs), 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% of all MZLs and typically presenting with lymph node–based disease without splenic or extranodal site involvement. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/29 UR - accessmedicine.mhmedical.com/content.aspx?aid=1180444347 ER -