Pathogenesis of classical and lymphocyte-predominant Hodgkin lymphoma

R Schmitz, J Stanelle, ML Hansmann… - Annual Review of …, 2009 - annualreviews.org
R Schmitz, J Stanelle, ML Hansmann, R Küppers
Annual Review of Pathology: Mechanisms of Disease, 2009annualreviews.org
Hodgkin and Reed-Sternberg (HRS) cells in classical Hodgkin lymphoma (HL) and
lymphocytic and histiocytic (L&H) cells in nodular lymphocyte–predominant HL (NLPHL) are
derived from germinal-center B cells. HRS cells have, however, largely lost their B cell
phenotype and aberrantly express markers and transcriptional regulators of other
hematolymphoid cell types. Deregulation of multiple signaling pathways and downstream
transcription factors, including receptor tyrosine kinases, nuclear factor–kappa B (NF-κB) …
Hodgkin and Reed-Sternberg (HRS) cells in classical Hodgkin lymphoma (HL) and lymphocytic and histiocytic (L&H) cells in nodular lymphocyte–predominant HL (NLPHL) are derived from germinal-center B cells. HRS cells have, however, largely lost their B cell phenotype and aberrantly express markers and transcriptional regulators of other hematolymphoid cell types. Deregulation of multiple signaling pathways and downstream transcription factors, including receptor tyrosine kinases, nuclear factor–kappa B (NF-κB), and Janus kinase/signal transducer and activator of transcription (JAK/STAT), is a further hallmark of HRS cells. These cells harbor genetic lesions that contribute to or cause increases in the activity of transcription factors of the NF-κB and STAT families. HRS cells are found within a mixed reactive cellular infiltrate and interact with these nonmalignant cells in a complex fashion that appears to be essential for HRS cell survival and proliferation. Less is known about the pathogenesis of L&H cells in NLPHL, but increases in the activity of receptor tyrosine kinases, NF-κB, and JAK/STAT have also been detected.
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