[Lymphomas of the ocular adnexa in Gougerot-Sjögren syndrome. Apropos of 4 cases].
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.
Chazerain
P
P
Service de Rhumatologie, Hôpital Bichat, Paris.
Meyer
O
O
Kaplan
G
G
Brissaud
P
P
Delmer
A
A
Zufferey
P
P
Kahn
M F
MF
D000368:Aged; D001172:Arthritis, Rheumatoid / Q000150:complications; D005134:Eye Neoplasms / Q000209:etiology*; D005260:Female; D006801:Humans; D008228:Lymphoma, Non-Hodgkin / Q000209:etiology*; D008875:Middle Aged; D011379:Prognosis; D012859:Sjogren's Syndrome / Q000150:complications*
Lymphomes des annexes de l'oeil au cours du syndrome de Gougerot-Sjögren.