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• W2890214671 abstract "e20109 Background: Patients (pts) with thymic epithelial tumour (TET) are more likely to have an autoimmune disease during their lives. The association of Good syndrome and hematological manifestations is not uncommon. Here we describe the clinical features and the treatment outcomes of ten pts affected by TET and Good syndrome complicated by Pure Red Cell Aplasia (PRCA). Methods: We retrospectively analyzed ten pts with TET and Good syndrome diagnosis, admitted to our Institution over a 10-years period, who developed PRCA. This series includes five men and five women (1:1 M: F ratio), with a median age of 47.5 years (41-65 years). Seven pts had an advanced disease at diagnosis according to Masaoka-Koga stage system: 4 pts IVA, 1 pt IVB, 1 pt III stage disease. Three patients had a II stage disease. Six patients underwent a first radical surgery. According to WHO 2004 classification, all the cases were thymoma: eight pts B2, 1 pt AB, 1 pt B3 thymoma. Good syndrome and PRCA were diagnosed in all pts. Immunoglobulin infusions at 500 mg/kg daily for 5 days were administrated for Good Syndrome control. High dose corticosteroids (1 mg/Kg/daily of prednisone) were used as first line treatment. The association with long acting somatostatin analogues in OctreoScan positive cases was started in five pts, among these, four received oral cyclosporine (2mg/Kg). The other five were treated with more aggressive immunosuppressive agents, such as alentuzumab and tacrolimus. Results: The only AB case reached a better outcome obtaining thymoma shrinkage and PRCA control by somatostatin analogues and prednisone association. Four pts had long thymoma and PRCA control by adding cyclosporine to long acting somatostatin analogs. Five pts treated with corticosteroids, alentuzumab ad tacrolimus died very soon due to rare viral infectious diseases. Conclusions: The histotype B2 is more likely associated to PRCA. In OctreoScan positive pts, the association of somatostatin analogues to oral cyclosporine seems to be the best treatment in terms of PRCA control and toxicity. In according to our experience more aggressive immunosuppressive agents such as alentuzumab and tacrolimus could be avoid due to the development of fatal infectious disease." @default.
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• W2890214671 date "2016-05-20" @default.
• W2890214671 modified "2023-09-23" @default.
• W2890214671 title "Successful treatment of good syndrome and pure red cell aplasia in thymic epithelial tumors." @default.
• W2890214671 doi "https://doi.org/10.1200/jco.2016.34.15_suppl.e20109" @default.
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