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Report: workshop on mediastinal grey zone lymphoma
Authors:Poppema Sibrand  Kluiver Joost L  Atayar Cigdem  van den Berg Anke  Rosenwald Andreas  Hummel Michael  Lenze Dido  Lammert Hetty  Stein Harald  Joos Stephan  Barth Thomas  Dyer Martin  Lichter Peter  Klein Uwe  Cattoretti Giorgio  Gloghini Annunziata  Tu Yuhai  Stolovitzky Gustavo A  Califano Andrea  Carbone Antonino  Dalla-Favera Ricardo  Melzner Ingo  Bucur Alexandra J  Brüderlein Silke  Dorsch Karola  Hasel Cornelia  Barth Thomas F E  Leithäuser Frank  Möller Peter
Affiliation:Department of Pathology and Laboratory Medicine, University Medical Center Groningen, University of Groningen De Brug, Rm 9.14, Hanzeplein 1, 9700 RB Groningen, The Netherlands. s.poppema@med.rug.nl
Abstract:
There are several indications that classical Hodgkin lymphoma (cHL) and at least a proportion of cases of Primary Mediastinal B cell Lymphoma (PMBL) are derived from B cells at similar stages of differentiation and share common pathogenic mechanisms. The first indication was the existence of mediastinal grey zone lymphomas as identified in the 4th International Symposium on HL, with clinical, histological and immunohistochemical features intermediate between cHL and PMBL. Second, both tumor types resemble a cell that is developmentally situated in-between the germinal center reaction and a plasma cell. Third, cHL and PMBL were found to have similar gene expression profiles, including the lack of immunoglobulin expression and low levels of B cell receptor signalling molecules, and the secretion of molecules like the chemokine TARC and the prominent expression of IL-13 receptors. Fourth, both entities were found to have common genomic aberrancies, notably in 2p15 and 9p24, the sites of the REL oncogene and the tyrosine kinase gene JAK2, respectively. Further comparison of both lymphoma types may provide further insight in the pathogenic mechanisms and allow the design of diagnostic algorithms to sort out the small number of so-called mediastinal grey zone lymphomas, that appear to be intermediate between PMBL and cHL.
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