MALT lymphoma revealed by parotidomegaly in patients with Sjögren’s syndrome

Abstract

The occurence of non-Hodgkin’s malignant lymphoma in patients with Gougerot-Sjögren Syndrome is not uncommon, and some predictive factors may point the practitioner towards the risk of possible degeneration.  We report two cases of patients followed for primary Sjögren’s syndrome who developed parotid MALT lymphoma. Results: Two patients aged 29 and 64 years followed for Sjögren’s syndrome. The first one was under azathioprine while the second was treated with corticosteroids. After a follow-up of 13 months and 9 years, respectively, patients consulted for aggravation of a pre-existing bilateral parotid swelling which became hard and sensitive and was accompanied with alteration of the general state of health. They were explored by a biological assessment and magnetic resonance imaging of the parotids. The parotid gland biopsy concluded that a MALT-type lymphoma turned into a large-cell B-lymphoma in one case and small-cell B-lymphoma in the other case. The treatment was based on chemotherapy with favorable evolution. Conclusion: The Sjögren’s syndrome is recognized as a pre-lymphomatous state on which MALT lymphomas from the marginal area are frequently added. Degeneration must be suspected in front of predictive lymphomatous transformation elements. Early management is essential to improve prognosis of connectivitis and lymphoma.