Primary renal marginal zone B lymphoma is extremely rare. The renal involvement by the lymphoma is most often a manifestation of a disseminated disease. We report the case of a 53-year-old patient who presented lower back pain and kidney failure. The renal biopsy showed a marginal zone B lymphoma type MALT and the PET scan showed bilateral renal involvement. A treatment based on rituximab combined with chemotherapy (cyclophosphamide, prednisone and oncovin) was started with good outcome.
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