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Objective: To investigate the clinical characteristics, diagnosis, treatment and prognosis of discordant lymphoma. Methods: The clinical data of one patient with discordant lymphoma at the PLA Strategic Support Force Characteristic Medical Center were retrospectively analyzed, and the related literatures were reviewed. Results: The patient was treated for thrombocytopenia and the examination showed splenomegaly. After hormone treatment, the platelet rebounded and thrombocytopenia occurred during hormone reduction. Splenectomy was performed. Postoperative pathological diagnosis of splenic marginal lymphoma was made and observed. Axillary lymph node enlargement occurred nine months later. Pathological diagnosis of diffuse large B-cell lymphoma was made by using lymph node biopsy, and the disease condition was alleviated after immunotherapy combined with chemotherapy. Conclusions Discordant lymphoma is rare and shows no special clinical manifestations. Its diagnosis should rely on pathological examination. Immunotherapy combined with chemotherapy may be more effective.