title: Hodgkin lymphoma: Pathology and biology.
abstract: The Hodgkin and Reed-Sternberg (HRS) tumor cells of classical Hodgkin lymphoma (HL), as well as the lymphocyte predominant (LP) cells of nodular lymphocyte predominant HL (NLPHL), are derived from mature B cells. However, HRS cells have largely lost their B-cell phenotype and show a very unusual expression of many markers of other hematopoietic cell lineages, which aids in the differential diagnosis between classical HL (cHL) and NLPHL and distinguishes cHL from all other hematopoietic malignancies. The bi- or multinucleated Reed-Sternberg cells most likely derive from the mononuclear Hodgkin cells through a process of incomplete cytokinesis. HRS cells show a deregulated activation of numerous signaling pathways, which is partly mediated by cellular interactions in the lymphoma microenvironment and partly by genetic lesions. In a fraction of cases, Epstein-Barr virus contributes to the pathogenesis of cHL. Recurrent genetic lesions in HRS cells identified so far often involve members of the nuclear factor-κB (NF-κB) and JAK/STAT pathways and genes involved in major histocompatibility complex expression. However, further lead transforming events likely remain to be identified. We here discuss the current knowledge on HL pathology and biology.
authors: Max-Delbrück-Center for Molecular Medicine, and Hematology, Oncology, and Tumor Immunology, Charité - Universitätsmedizin Berlin, Berlin, Germany.
authors: Stephan
authors: S
authors: Mathas
authors: Dr. Senckenberg Institute of Pathology, University of Frankfurt, Medical School, Frankfurt/Main, Germany.
authors: Sylvia
authors: S
authors: Hartmann
authors: Institute of Cell Biology (Cancer Research), Medical Faculty, University of Duisburg-Essen, Essen, Germany. Electronic address: ralf.kueppers@uk-essen.de.
authors: Ralf
authors: R
authors: Küppers
mesh_terms: D002450:Cell Communication; D015972:Gene Expression Regulation, Neoplastic; D006689:Hodgkin Disease / Q000235:genetics / Q000378:metabolism* / Q000473:pathology*; D006801:Humans; D016328:NF-kappa B / Q000378:metabolism; D010641:Phenotype; D016539:Reed-Sternberg Cells / Q000378:metabolism; D015398:Signal Transduction / Q000235:genetics
chemical_list: D016328:NF-kappa B
keywords: Germinal center; Hodgkin lymphoma; LP cell