RT Book, Section A1 Becnel, Melody A1 Samaniego, Felipe A2 Kantarjian, Hagop M. A2 Wolff, Robert A. A2 Rieber, Alyssa G. SR Print(0) ID 1190832633 T1 Marginal Zone and Other Small Cell Lymphomas T2 The MD Anderson Manual of Medical Oncology, 4e YR 2022 FD 2022 PB McGraw Hill Education PP New York, NY SN 9781260467642 LK accessmedicine.mhmedical.com/content.aspx?aid=1190832633 RD 2024/03/29 AB KEY CONCEPTSMarginal zone lymphoma (MZL) is an indolent B-cell non-Hodgkin lymphoma composed of three subtypes: extranodal (mucosal-associated lymphoid tissue [MALT] lymphoma), splenic, and nodal.MALT lymphoma, the most common subtype, is composed of gastric and nongastric MALT and is often associated with infectious organisms such as Helicobacter pylori.Nongastric MALT is often associated with inflammatory conditions such as Sjögren syndrome and Hashimoto thyroiditis.Translocations well characterized in MALT lymphomas include t(11;18), t(14;18), t(1;14), and t(3;14). No unique recurrent chromosomal abnormalities are noted in nodal MZL. MYD88 L265P mutations (as seen in Waldenström macroglobulinemia) have been identified in 10% to 20% of cases of splenic MZL.WHO classification restricts small lymphocytic lymphoma (SLL) to tumors involving lymph nodes with the same B-cell immunophenotype as CLL without leukemic involvement and considers SLL to be the nodal or tissue counterpart of chronic lymphocytic leukemia (CLL).Abnormalities of the p53 or MYC genes correlate with increased risk of histologic transformation (Richter syndrome) and poorer prognosis in CLL/SLL.