Diffuse Large B-Cell Lymphoma as a Sequela of Sjögren’s Syndrome: A Case Report

ABSTRACT

We present a case of a 45-year-old woman with primary Sjögren's syndrome, who in 2005, one year after the onset of the systemic autoimmune disease developed chronic lymphocytic leukaemia, which eventually transformed - denoted as Richter’s transformation - into diffuse large B-cell lymphoma in 2006. She had recurrent lymphadenopathy on the neck region from 2002. In September 2005 anaemia and elevated LDH enzyme levels were detected with persistent subfebrility and lymphatic node enlargement on her neck and axillary region. Chest and abdominal CT, biopsy from the lymphoid glands and crista biopsy were performed and B-CLL was diagnosed, therefore chlorambucil treatment was administered. After two cycles of chlorambucil the pulmonary involvement extended, the patient had high fever and the abdominal CT showed the appearance of multiple hypodens lesions in the liver. Based on the histological evaluation of the hypodens lesions of the liver, the diagnosis of diffuse large B-cell lymphoma has been established, therefore 8 cycles of R-CHOP treatment were administered. The patient has been in complete remission since then.

Conclusion: The main message of this case report is that lymphadenopathy, the decrease of serum immunoglobulin levels and autoantibody titres, as well as the predominance of monoclonal gammopathy point to the possibility of malignant transformation in patients with Sjögren's syndrome.