A 49-year-old woman, with no relevant personal or familial history, presented to the Emergency Department with upper left abdominal pain, early satiety, night sweats and an 8% weight loss within the previous two months. She reported no fever. On admission she was pale, tachycardic and with palpable splenomegaly, six centimetres below the left costal margin. Blood tests revealed a normochromic and normocytic anaemia (Hb 9 g/dl), thrombocytosis (445 x 109/l, reference range 150-400 x 109/l), LDH 892 UI/l (reference range 85-227 UI/l), erythrocyte sedimentation rate (ESR) of 101 mm/h, C-reactive protein (CRP) of 17.30 mg/dl (reference range < 0.60 mg/dl) and hypoalbuminemia (3.1 g/dl). The magnetic resonance imaging (MRI, Fig. 1) of the abdomen showed a multinodular splenomegaly of 15x11x17 (Fig. 1B, arrow).
Serial blood cultures were negative as well as tests performed to exclude several infectious diseases such as infectious mononucleosis, cytomegalovirus, brucellosis, schistosomiasis, echinococcosis, human immunodeficiency virus, hepatitis B and C and tuberculosis. Myelogram and bone marrow biopsy showed no significant abnormalities - no leishmanias. There was no evidence of involvement of other organs, as confirmed by F-18 fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT). An ultrasound-guided biopsy of the spleen confirmed a primary splenic DLBCL, with non-germinal centre B-cell-like (non-GCB) immunophenotype (Fig. 2 and 3), configuring a stage I disease. Her International Prognostic Index (IPI) score was 1 (low risk).
The patient started on first-line chemotherapy with conventional R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone) and experimental tafasitamab, a CD19-directed cytolytic antibody, approved for treatment of refractory, transplant-ineligible, DLBCL. At two years of follow-up the patient maintains complete remission.