Sjogren's Syndrome: Lymphoma predisposition coupled with a reduced frequency of t(14;18) translocations in blood lymphocytes

Sjogren's syndrome (SS) is a systemic autoimmune disorder with a strong tum or predisposition (a 44-fold elevated incidence of non-Hodgkin's lymphoma h as been reported). By polymerase chain reaction analysis of t(14; 18), a ke y lymphomagenic event in peripheral blood lymphocytes, we found a lower fre quency 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 auto antibodies (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 deve lopment may be explained by a constitutive deficiency in V(D)J recombinase leading to autoimmunity and increased lymphoproliferation. Mol. Carcinog. 2 4:226-231, 1999. (C) 1999 Wiley-Liss, Inc.