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A 52-year-old woman with Sjögren's syndrome and immunologic thrombocytopenic purpura was found to have a mediastinal tumor. Preoperative examination revealed the patient suffered from a severe thrombocytopenia and even has a rare blood group: O D+ (D+c+c+E+E+). Resection of the mediastinal tumor using Video-Assisted Thoracic Surgery (VATS) after thoroughly prepared including thrombocyte transfusion. Histopathologic examination and immunohistochemistry of the thymus tumor were both consistent with Mucosa-Associated Lymphoid Tissue (MALT) lymphoma. This rare case suggests that thymic MALT lymphoma can develop with an autoimmunue disease such as Sjögren's syndrome, surgical resection of thymic tumor should be performed after careful preoperative prepration.