| Objective:To improve the knowledge in primary splenic NK/T cell lymphoma.Methods:The clinical features, histopathologic characteristics, treatment and prognosis of 1patient with primary splenic NK/T cell lymphoma with EB virus infected was analyzed,and the related literatures were reviewed.Results:A 40-year-old man was admitted to shanxi dayi hospital with a 6-year history of splenomegaly, and his splenic grown rapidly with fever for 7 months, with a highly EBV-DNA copy in his peripheral blood reached 5.2 x 109 copy/ml, without superficial and enterocoelia lymphadenopathy, without involvement in lungs and liver.Bone marrow smear found 6% atypical lymphoma cells.He was definitely diagnosed as splenic NK/T cell lymphoma by splenectomy.After the splenectomy for 1 month, the patient was detected concrete masses in his lungs and liver by a computed tomography(CT), and complicated with hemophagocytic syndrome, disseminated intravascular coagulation,hemorrhage of digestive tract and respiratory failure. The patient received active supporting therapy and combined therapy of etoposide 50mg/day, dexamethasone15mg/day, intravenous immunoglobulin 12.5g/day and ganciclovir 500mg/day for 10 days and had been partial remission rapidly. The EBV-DNA copy was progressively decreased.Conclusion:Primary splenic NK/T cell lymphoma is rare, which is characterized by progressively enlarging of spleen with fever, highly invasive, hemophagocytic syndrome,multiple organ failure, poor prognosis.EB viral copy has close relationship with disease’s occurrence and development. Etoposide combined with dexamethasone and ganciclovir had been proved playing a significant role in eliminating EB virus and relieving primary splenic NK/T cell lymphoma complicated with hemophagocytic syndrome. However,active chemotherapy is still necessary for patients received remission. |
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