Exceptionally Rare Lymphoma Entity: Primary Uterine Marginal Zone Lymphoma in a Patient with Rheumatoid Arthritis

Primary MALT non-Hodgkin’s lymphoma (NHL) of the uterus is an extremely rare entity. Both etiology and pathogenesis of these primary extranodal NHL lymphomas are unknown. Some researchers suppose a possible association between chronic inflammation, autoimmune diseases and lymphomas. We report a case of MALT uterine lymphoma of a 73-year-old woman. Postmenopausal vaginal bloody discharge was the leading symptom. Fractionated curettage was made and hystological analysis proven the MALT lymphoma diagnosis. Because of the low incidence of female genital tract lymphomas, there is no evidence-based consensus on its treatment. Eight cycles of rituximab plus CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone; R-CHOP) chemoimmunotherapy was administered. The patient is disease-free after 20 months of therapy. R-CHOP is a good and tolerable treatment option in elderly patients. Further case reports and multicenter analysis can help to evaluate the long-term results of chemoimmunotherapy. Keywords: MALT uterine lymphoma, primary extranodal lymphoma, Rituximab-based therapy, rheumatoid arthritis linked lymphoma