Report: workshop on mediastinal grey zone lymphoma.
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.
Poppema
Sibrand
S
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
Kluiver
Joost L
JL
Atayar
Cigdem
C
van den Berg
Anke
A
Rosenwald
Andreas
A
Hummel
Michael
M
Lenze
Dido
D
Lammert
Hetty
H
Stein
Harald
H
Joos
Stephan
S
Barth
Thomas
T
Dyer
Martin
M
Lichter
Peter
P
Klein
Uwe
U
Cattoretti
Giorgio
G
Gloghini
Annunziata
A
Tu
Yuhai
Y
Stolovitzky
Gustavo A
GA
Califano
Andrea
A
Carbone
Antonino
A
Dalla-Favera
Ricardo
R
Melzner
Ingo
I
Bucur
Alexandra J
AJ
Brüderlein
Silke
S
Dorsch
Karola
K
Hasel
Cornelia
C
Barth
Thomas F E
TF
Leithäuser
Frank
F
Möller
Peter
P
D001402:B-Lymphocytes / Q000378:metabolism / Q000473:pathology; D002869:Chromosome Aberrations; D004493:Education; D015973:Gene Expression Regulation, Leukemic; D006689:Hodgkin Disease / Q000235:genetics / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D016393:Lymphoma, B-Cell / Q000235:genetics / Q000473:pathology / Q000503:physiopathology*; D008479:Mediastinal Neoplasms / Q000235:genetics / Q000473:pathology / Q000503:physiopathology*