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Sjögren's syndrome: Lymphoma predisposition coupled with a reduced frequency of t(14;18) translocations in blood lymphocytes
Author(s) -
Henriksson Gunnel,
Brant Marta,
Sandor Zoltan,
Manthorpe Rolf,
Bredberg Anders
Publication year - 1999
Publication title -
molecular carcinogenesis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.254
H-Index - 97
eISSN - 1098-2744
pISSN - 0899-1987
DOI - 10.1002/(sici)1098-2744(199903)24:3<226::aid-mc9>3.0.co;2-6
Subject(s) - biology , immunology , autoantibody , autoimmunity , chromosomal translocation , lymphoma , population , recombinase , genetics , antibody , gene , recombination , medicine , environmental health
Sjögren's syndrome (SS) is a systemic autoimmune disorder with a strong tumor predisposition (a 44‐fold elevated incidence of non‐Hodgkin's lymphoma has been reported). By polymerase chain reaction analysis of t(14;18), a key lymphomagenic event in peripheral blood lymphocytes, we found a lower frequency in a subset of 12 SS patients positive for SS‐A/SS‐B autoantibodies than in 21 healthy subjects and 20 SS patients lacking these SS marker autoantibodies ( P < 0.001). All 14 mutants sequenced displayed signs typical of V(D)J recombinase activity. This perplexing result of a low rate of t(14;18) in a population strongly predisposed to t(14;18)‐associated tumor development may be explained by a constitutive deficiency in V(D)J recombinase leading to autoimmunity and increased lymphoproliferation. Mol. Carcinog. 24:226–231, 1999. © 1999 Wiley‐Liss, Inc.