Case Report: PDF OnlyPrimary Splenic Lymphoma Associated with Hemophagocytic Lymphohistiocytosis Complicated with Splenic RuptureHan, Shao-Mina; Teng, Chieh-Lina, b; Hwang, Guang-Yuhb; Chou, Guanc; Tsai, Che-And, * Author Information aDivision of Hematology/Oncology, Department of Internal Medicine, Taichung Veterans General Hospital, Taichung, Taiwan, R.O.C. bDepartment of Life Science, Tunghai University, Taichung, Taiwan, R.O.C. cDepartment of Pathology, Taichung Veterans General Hospital, Taichung, Taiwan, R.O.C. dDivision of Infection, Department of Internal Medicine, Taichung Veterans General Hospital, Taichung, Taiwan, R.O.C. *Correspondence to: Dr Che-An Tsai, Division of Infection, Department of Internal Medicine, Taichung Veterans General Hospital, 160, Section 3, Chung-Kang Road, Taichung 407, Taiwan, R.O.C E-mail: [email protected] Received: August 28, 2007; • Accepted: January 14, 2008. Journal of the Chinese Medical Association 71(4):p 210-213, April 2008. | DOI: 10.1016/S1726-4901(08)70106-0 Metrics Abstract Primary splenic lymphoma (PSL) is a rare disease with ambiguous definition, comprising less than 1% of non-Hodgkin's lymphoma. Even rarer is PSL combined with hemophagocytic lymphohistiocytosis (HLH), which has presentations of fever, cytopenia, hepatosplenomegaly, hyperferritinemia, and phagocytosis of hematopoietic cells in the reticuloendothe-lial system. We report the case of a 77-year-old man who presented with HLH initially. Refusing diagnostic splenectomy, he received chemotherapy. Spontaneous splenic rupture occurred after chemotherapy. In the following emergency operation, PSL was diagnosed. He received another 5 courses of chemotherapy with the R-CNOP regimen (rituximab, cyclophosphamide, mitoxantrone, vincristine, prednisolone). Now he has no residual or relapsed disease. Diagnostic splenectomy for adult HLH patients without definite etiologies may play an important role. [J Chin Med Assoc 2008;71(4):210–213] © 2008 by Lippincott Williams & Wilkins, Inc.