Clinical Report Of 12 Cases Of Thymoma Associated With Pure Red Cell Aplasia

Objective:Thymoma associated with pure red cell aplasia(PRCA)is an infrequent syndrom.Its physiopathologic mechanism is not clear.There is no standard treatment for thymoma associated with pure red cell aplasia.We aim to investigate the clinical characteristics,diagnosis and treatment of thymoma associated with PRCA,in order to make a further clinical understanding of thymoma associated with PRCAMethod:Analyze the clinical data and telephone follow-up from 12 patients of Shandong Provincial Hospital in recent 10 years who were diagnose thymoma associated with PRCA Result:In this series of cases,there are 5 male cases and 7 female cases,age range from 42 to 71,average age is 58.17,median age is 58.5.All of these 12 patients went to see their doctor for the the symptoms of anemia such as paleness,dizziness and weakness.8 of them have thymectomy via media sternotomy,2 of them have it via right thoracic incision and 2 of them have it via Video-assisted Thoracoscopic Surgery(VATS).No one died after the thymectomy.Postoperative histopathology showed:3 cases of AB type,3 cases of B1 type,4 cases of B2 type,2 case of B3 type.Masaoka standard was performed in this group,including 8 cases in stage Ⅰ,3 cases in stage Ⅱ and 1 case in stage Ⅳ.All 12 patients were followed up by telephone,11 patients were followed up from June 2016 to December 2016,1 patient loss to follow up.At the end of follow-up,7 patients achieved complete cure,3 patients died and 1 patient remain transfusion dependent.Of the patients who achieved complete cure,2 cases got cure after the surgery and receive no other treatment,1 treated with prednisone alone,1 treated with antithymocyte Globulin(ATG)and prednisone,and the remaining 3 treated with ATG and cyclosporine.1 patient remain transfusion independent for a year after treated with cyclosporine,but PRCA relapse when reduced the dosage for drug toxicity.3 patients died,2 of them died of infection(1 case died of empyema,1 case died of bronchiectasis associated with pneumonia),and 1 died of metastatic thymoma.Conclusions:Thymoma is a common mediastinal tumor,but thymoma associated with PRCA is relatively rare.The prognosis of thymoma with PRCA depends mainly on the treatment of PRCA.PRCA caused by thymoma may be related to immune factors.Thymectomy is the primary treatment of thymoma associated with PRCA,but only a few patients get complete cure after thymectomy.Most patients need other treatment after surgery,such as steroids,cyclosporine and ATG.