Evidence that Hodgkin and Reed-Sternberg cells in Hodgkin disease do not represent cell fusions.
In most cases, Hodgkin and Reed-Sternberg (HRS) cells of classical Hodgkin disease (HD) carry rearranged immunoglobulin (Ig) genes and thus derive from B cells. In rare cases, HRS cells originate from T cells. However, based on the unusual immunophenotype of HRS cells, often showing coexpression of markers typical for different hematopoietic lineages, and the regular detection of numerical chromosomal abnormalities, it has been speculated that HRS cells might represent cell fusions. Five cases of HD with 2 rearranged IgH alleles were analyzed for the presence of additional IgH alleles in germline configuration as a potential footprint of a cell fusion between a B and a non-B cell. Similarly, one case of T-cell-derived HD with biallelic T-cell receptor beta (TCRbeta) rearrangements was studied for the presence of unrearranged TCRbeta alleles. In none of the 6 cases was evidence for additional IgH (or TCRbeta) alleles obtained, strongly arguing against a role of cell fusion in HRS cell generation.
Küppers
R
R
Institute for Genetics and the Department of Internal Medicine I, University of Cologne, Cologne, Germany. rkuppers@mac.genetik.uni-koeln.de
Bräuninger
A
A
Müschen
M
M
Distler
V
V
Hansmann
M L
ML
Rajewsky
K
K
D002459:Cell Fusion; D015326:Gene Rearrangement, B-Lymphocyte, Heavy Chain; D015333:Gene Rearrangement, beta-Chain T-Cell Antigen Receptor; D018095:Germ-Line Mutation; D006689:Hodgkin Disease / Q000235:genetics*; D006801:Humans; D007128:Immunoglobulin Fragments; D016539:Reed-Sternberg Cells / Q000473:pathology*
D007128:Immunoglobulin Fragments