title: Cellular origin of nodular lymphocyte-predominant Hodgkin's lymphoma: immunophenotypic and molecular studies.
abstract: The lymphocyte-predominant (LP) type of Hodgkin's lymphoma (HL) is a unique subtype with characteristic tumor cells (lymphocytic and/or histiocytic [L&H] cells) in associated macronodular structures that resemble progressively transformed germinal centers (PTGCs). Immunohistochemical studies have provided strong evidence that L&H cells are of B-cell lineage and recent molecular studies suggested they are transformed centroblasts. A major clonal population is detectable at presentation, with the immunoglobulin heavy-chain gene often showing evidence of continued somatic hypermutation. In developed nations, Epstein-Barr virus (EBV) is infrequently associated with L&H cells and is probably not involved in the pathogenesis of this disease. L&H cells are frequently surrounded by CD3+, CD4+, CD57+, and CD40L- T cells, but the significance of this T-cell rosetting is unclear. LPHL may be associated with concurrent or subsequent large B-cell lymphoma, and there is evidence of a clonal relationship between the two entities. LPHL may also have nodules or large areas that resemble histiocyte-rich B-cell lymphoma (HRBCL). It is likely that at least some cases of HRBCL arise from LPHL. The same may be true of T-cell-rich B-cell lymphoma. Little is known about cytogenetic abnormalities, the cytokine expression profile, and the expression of several functionally important molecules that have been demonstrated in the Reed-Sternberg (RS) cells of classical HL. The challenge for the future is to obtain a more comprehensive molecular profile of L&H cells and their associated T lymphocytes, so as to provide a framework for eventual elucidation of the pathogenesis of this type of HL.
authors.affiliation: Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha 68198-3135, USA.
authors.forename: W C
authors.initials: WC
authors.lastname: Chan
mesh_terms: D001402:B-Lymphocytes / Q000473:pathology; D019070:Cell Lineage; D006689:Hodgkin Disease / Q000150:complications / Q000209:etiology* / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D008214:Lymphocytes / Q000473:pathology; D016393:Lymphoma, B-Cell; D008224:Lymphoma, Follicular / Q000209:etiology / Q000473:pathology*; D008228:Lymphoma, Non-Hodgkin / Q000150:complications