Abstract:
Gougerot-Sj?gren's syndrome (GSS) is a connective tissue disease characterised by lymphocytic infiltration of the exocrine glands and polyclonal activation of B lymphocytes. The incidence of malignant lymphomas, Waldenstr?m's disease and benign monoclonal gammopathies is high. However, progression to multiple myeloma (MM) is exceptional. We report the case of a 54-year-old female patient with primary GSS who developed symptomatic IgA Kappa MM 7 years later, treated with VDT-type multidrug therapy and an autologous haematopoietic stem cell transplant. Biological reassessment at the end of treatment showed complete remission, and the patient received maintenance treatment with lenalidomide. After one year of maintenance treatment, she developed non-metastatic breast cancer, which was treated with breast-conserving surgery and adjuvant chemo-radiotherapy.
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