ABSTRACT
This chapter examines data on the epidemiology, clinical features, staging, and treatment of the lymphomas. Splenic marginal zone lymphoma (SMZL) is considered as a distinct entity among non-Hodgkin’s lymphomas, after the proposal as a provisional entity in the Revised European–American Classification of Lymphoid Neoplasms classification, and the subsequent recognition as a distinct entity in the World Health Organization classification. In the spleen, the SMZL is characterized by a micronodular expansion of small B lymphocytes inside the white pulp, associated with variable degree of involvement of the splenic red pulp. The marginal zone is located around lymphoid follicles of secondary lymphoid organs, including lymph nodes, spleen, and extranodal mucosa-associated lymphoid tissue, such as Peyer’s patches of small intestine. The SMZL is a marginal zone memory B cell, claimed in most cases to have a postgerminal origin, as demonstrated by the study of somatic mutations in Ig heavy-chain variable region genes.
