[Lymphoproliferative disorders in patients with Sjögren's syndrome--Vg gene rearrangement and expression of bcl-2 protein].
Sjögren's syndrome (SS) is a unique disease which develops a high incidence of lymphoproliferative disorders (LPD) such as monoclonal gammopathy or malignant lymphoma. In order to elucidate the mechanism responsible for the emergence of monoclonal B cell proliferation at the site of a chronic autoimmune reaction, the rheumatoid factor (RF) related germline gene Vg and expression of the bcl-2 gene in the lymphoepithelial lesion (LEL) were studied. Predominant usage of the Vg or Vg-like genes was found in the peripheral blood leukocytes in SS patients and abundant expression of bcl-2 protein in the lymphocytes in and around the LEL were observed. These results suggest that RF clones were activated resulting into monoclonal proliferation and overexpression of bcl-2 protein play a role allowing the cell to escape a apoptotic cell death resulting into the increased risk of monoclonal proliferation in SS patients.
Intractable Disease Section, Kanazawa Medical University.
S
S
Sugai
S
S
Takeshita
Y
Y
Ogawa
Y
Y
Masaki
T
T
Doumiki
Y
Y
Takeuchi
D005260:Female; D015321:Gene Rearrangement; D006801:Humans; D008214:Lymphocytes / Q000378:metabolism; D008223:Lymphoma / Q000209:etiology*; D008232:Lymphoproliferative Disorders / Q000209:etiology*; D008297:Male; D011518:Proto-Oncogene Proteins / Q000378:metabolism*; D019253:Proto-Oncogene Proteins c-bcl-2; D012217:Rheumatoid Factor / Q000235:genetics*; D012859:Sjogren's Syndrome / Q000150:complications*
D011518:Proto-Oncogene Proteins; D019253:Proto-Oncogene Proteins c-bcl-2; D012217:Rheumatoid Factor