Abstract. Fever is a common clinical presentation of a number of diseases. A sustained unexplained fever >38.3˚C lasting for >3 weeks without an established diagnosis despite intensive diagnostic evaluation is referred to as fever of unknown origin (FUO). FUO remains a clinical challenge for physicians, as it may be attributed to a wide range of disorders, mainly infections, malignancies, non-infectious inflammatory diseases and miscellaneous diseases. We herein report the case of a 59-year-old male patient who presented with prolonged unexplained fever and was found to have a diffusely enlarged hypermetabolic spleen, as shown on 18 F-fluorodeoxyglucose positron emission tomography/computed tomography examination. Following splenectomy, histopathological examination revealed primary splenic lymphoma (PSL) of B-cell origin. The patient received 6 courses of systemic chemotherapy with rituximab, etoposide, cyclophosphamide, doxorubicin, vincristine and prednisone (R-ECHOP regimen) and responded well to treatment. Thus, in patients with FUO and splenomegaly, the possibility of PSL should be taken into consideration.
IntroductionThe term ʻfever of unknown originʼ (FUO) was first introduced by Petersdorf and Beeson in 1961 based on an analysis of 100 cases, and it was defined as recurrent fever >38.3˚C, lasting for >3 weeks, remaining undiagnosed after 1 week of in-hospital evaluation (1). Several decades later, the criteria of FUO diagnosis have changed and it is currently defined by lack of a definitive diagnosis after appropriate inpatient or outpatient evaluation (2). In view of the patient's clinical circumstances and underlying immune status, FUO was categorized into classic, nosocomial, neutropenic and human immunodeffiency virus (HIV)-associated FUO by Durack and Street in 1991 (3). The etiologies of classic FUO mainly include infections, malignancies, non-infectious inflammatory diseases and miscellaneous causes, while some cases remain undiagnosed (4,5).Primary splenic lymphoma (PSL) is a rare malignant lymphoma with an incidence of ~1% among patients with non-Hodgkin lymphoma (NHL) (6), although the spleen is involved in approximately half of the cases of Hodgkin's disease and one-third of NHLs as part of systemic disease (7,8). Dasgupta et al (9) strictly defined PSL as lymphoma originating in the spleen and limited to the spleen and splenic hilum, without invasion of other sites, with an interval of ≥6 months prior to the appearance of lymphoma elsewhere.We herein present the case of a 59-year-old male patient with diffuse large B-cell PSL, who only exhibited sustained fever, without other remarkable complaints. To investigate the cause of the FUO, fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography ( 18 F-FDG-PET/CT) examination was performed and revealed a diffusely enlarged hypermetabolic spleen. Following diagnostic splenectomy, the histopathological diagnosis was diffuse large B-cell primary splenic NHL.
Case reportA 59-year-old male patient presented with a history of fe...