[Lymphomas of the ocular adnexa in Gougerot-Sjögren syndrome. Apropos of 4 cases]

Ann Med Interne (Paris). 1995;146(4):223-5.
[Article in French]

Abstract

The risk of malignant B cell lymphoma is increased in Sjögren's syndrome (SS). Orbital localization seems infrequent. We report 4 cases of malignant lymphoma (ML) occurring in 4 women aged 47 to 77 years, with primary SS in 3 cases, located to the conjunctiva in 2 cases, the lacrymal gland in 1 case and the eyelid in 1 case. The interval between the diagnosis of SS and orbital ML varied from 6 months to 15 years. All 4 lymphomas were of the B cell type, low histopathologic grade, with monoclonal gammopathy in 1 case. Extraocular lymphoma was initially present in 1 case. ML remained localized in 2 cases with a follow-up of 4 and 6 years. Two patients treated by excisional biopsy alone are in complete remission 3 and 6 years later. The 2 other patients treated with orbital radiotherapy and chemotherapy died rapidly (transformation into a high grade malignancy in 1 case). We conclude that clinical, immunopathologic features, as well as prognosis and treatment of ocular adnexa ML in SS are similar to those of primary ML without SS.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Aged
  • Arthritis, Rheumatoid / complications
  • Eye Neoplasms / etiology*
  • Female
  • Humans
  • Lymphoma, Non-Hodgkin / etiology*
  • Middle Aged
  • Prognosis
  • Sjogren's Syndrome / complications*