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Hodgkin Lymphoma pp 33–48Cite as

Pathology and Molecular Pathology of Hodgkin Lymphoma

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Part of the book series: Hematologic Malignancies ((HEMATOLOGIC))

Abstract

Hodgkin lymphoma (HL) comprises two disease entities: nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) and classical Hodgkin lymphoma (cHL). The new WHO classification maintains the subdivision of cHL into histological subtypes: nodular sclerosis cHL (NSCHL), mixed cellularity cHL (MCCHL), lymphocyte-depleted cHL (LDCHL), and lymphocyte-rich cHL (LRCHL). The tumor cells in NLPHL are termed lymphocyte predominant (LP) cells, whereas in cHL, Hodgkin and Reed–Sternberg (HRS) cells form the neoplastic population. HRS cells appear to be derived from germinal center (GC) B cells that normally would have undergone apoptosis, whereas LP cells originate from GC B cells that were positively selected. HRS cells have a downregulated expression of most B cell-typical genes and express multiple genes of other hematopoietic cell types. Constitutive activity of multiple signaling pathways is a hallmark of HRS cells. These pathways and transcription factors include receptor tyrosine kinases (RTK), NF-kB, Janus kinase/signal transducer and activator of transcription (JAK/STAT), and PI3 kinase, which contribute to the survival and proliferation of the malignant cells. A number of recurrent genetic lesions have been identified in HRS cells, and these often involve members of the NF-kB or JAK/STAT signaling pathways. We know less about the pathogenesis of LP cells in NLPHL, but constitutive activity of NF-kB, the JAK/STAT pathways, and of the BCL6 transcription factor appears to be involved.

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Authors and Affiliations

  1. Institute of Pathology, University of Würzburg, Würzburg, Germany

    Andreas Rosenwald

  2. Institute of Cell Biology (Cancer Research), University of Duisburg-Essen, Medical School, Virchowstr. 173, 45122, Essen, Germany

    Ralf Küppers

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  1. Klinik I für Innere Medizin, Universität Köln, Joseph-Stelzmann-Str. 9, Köln, 50924, Germany

    Andreas Engert

  2. Dept. Medicine, Stanford University, Stanford, 94305-5479, California, USA

    Sandra J. Horning

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Rosenwald, A., Küppers, R. (2011). Pathology and Molecular Pathology of Hodgkin Lymphoma. In: Engert, A., Horning, S. (eds) Hodgkin Lymphoma. Hematologic Malignancies. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-12780-9_3

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