Non-Hodgkin's lymphoma associated with primary Sjögren's syndrome.
OBJECTIVE
To evaluate the prevalence, the incidence and clinical presentation of non-Hodgkin's lymphoma associated with primary Sjögren's syndrome.


METHODS
Sixty-two patients with primary Sjögren's syndrome were analyzed retrospectively in an open investigation.


RESULTS
Of 62 patients with primary Sjögren's syndrome, 4 of them (6.4%) developed non-Hodgkin's lymphoma (6.9 cases per 1000 per year). All of them were women. Non-Hodgkin's lymphoma always developed after the onset of primary Sjögren's syndrome with a time interval ranging from 3 to 27 years. Pathological findings showed two diffused mixed small and large cell cleaved lymphomas and two diffused large cell cleaved lymphomas. Three cases had extra-nodular localizations. All of these 4 patients are still alive and in complete remission 2 to 8 years after the diagnosis of non-Hodgkin's lymphoma.


CONCLUSIONS
This study confirms the association of non-Hodgkin's lymphoma in primary Sjögren's syndrome. These non-Hodgkin's lymphomas frequently had extra-nodal localizations. Good sensitivity to treatment, when necessary, provided good prognosis.
Pariente
D
D
Service d'Immuno-Rhumatologie, Centre Gui-de-Chauliac, Hôpital Saint-Eloi, Montpellier, France.
Anaya
J M
JM
Combe
B
B
Jorgensen
C
C
Emberger
J M
JM
Rossi
J F
JF
Sany
J
J
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001327:Autoimmune Diseases / Q000150:complications* / Q000453:epidemiology / Q000473:pathology; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D015994:Incidence; D008228:Lymphoma, Non-Hodgkin / Q000453:epidemiology / Q000209:etiology* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012189:Retrospective Studies; D012859:Sjogren's Syndrome / Q000150:complications* / Q000453:epidemiology / Q000473:pathology