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

✍ Scribed by Gunnel Henriksson; Marta Brant; Zoltan Sandor; Rolf Manthorpe; Anders Bredberg

• Publisher: John Wiley and Sons
• Year: 1999
• Tongue: English
• Weight: 141 KB
• Volume: 24
• Category: Article
• ISSN: 0899-1987
• DOI: 10.1002/(sici)1098-2744(199903)24:3<226::aid-mc9>3.0.co;2-6

✦ Synopsis

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.