title: Primary splenic lymphoma associated with hemophagocytic lymphohistiocytosis complicated with splenic rupture.
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 reticuloendothelial 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.
authors.affiliation: Division of Hematology/Oncology, Department of Internal Medicine, Taichung Veterans General Hospital, Taichung, Taiwan, R.O.C.
authors.forename: Shao-Min
authors.initials: SM
authors.lastname: Han
authors.forename: Chieh-Lin
authors.initials: CL
authors.lastname: Teng
authors.forename: Guang-Yuh
authors.initials: GY
authors.lastname: Hwang
authors.forename: Guan
authors.initials: G
authors.lastname: Chou
authors.forename: Che-An
authors.initials: CA
authors.lastname: Tsai
mesh_terms: D000368:Aged; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications*; D016403:Lymphoma, Large B-Cell, Diffuse / Q000150:complications* / Q000628:therapy; D008297:Male; D013160:Splenic Neoplasms / Q000150:complications* / Q000628:therapy; D013161:Splenic Rupture / Q000209:etiology*