LAUSR

A 68yearold woman presented to the Emergency Department with collapse, on a background of 4 months of intermittent headaches, weight loss and anorexia. Her past medical history was significant only for type 2 diabetes and Raynaud's phenomenon. There was no history of trauma or recent travel. On examination, she was haemodynamically compromised and peritonitic with severe leftsided tenderness. Laboratory tests showed a haemoglobin concentration of 76 g/l (normal two weeks earlier), and a markedly elevated lactate of 7.4 mmol/l. Wholebody computed tomography demonstrated marked splenomegaly (17.5 cm bipolar length) with spontaneous splenic rupture and haemoperitoneum (left image), bilaterally enlarged adrenal glands, and an incidental cerebral spaceoccupying lesion. Following resuscitation, she had an uncomplicated emergency splenectomy; intraoperatively, the spleen was enlarged and had a friable and fragile character. Postoperatively the patient was intermittently drowsy, with worsening headaches and functional decline. Magnetic resonance imaging of her head confirmed a 2.6 cm heterogeneouslyenhancing mass lesion in the left parietooccipital region with surrounding white matter oedema (right image). Blood cultures were negative, and a full viral screen including cytomegalovirus (CMV), Epstein– Barr virus (EBV) and human immunodeficiency virus (HIV) was unremarkable. Splenic histology demonstrated diffuse proliferation of pleomorphic lymphoid cells expressing CD20, CD79a, CD5 and MUM1 with variable BCL2 expression and a Ki67 proliferative index of 80%. A diagnosis of CD5+ diffuse large Bcell lymphoma (DLBCL) with central nervous system (CNS) involvement was made. The patient was treated with combination immunochemotherapy targeting both systemic and CNS disease, with a plan for consideration of autologous stem cell transplantation as consolidation therapy. Whilst 1% of splenic ruptures are spontaneous (the majority being traumatic in origin), there is a 10– 15% associated mortality with a large majority of cases having a pathologically abnormal spleen. The causes are diverse, but most often rupture is secondary to haematological malignancy, infection (commonly primary EBV infection or malaria), rheumatological disease (vasculitis, systemic lupus erythematosus) or amyloidosis, although drugrelated and iatrogenic cases can occur. Adding to a handful of cases secondary to DLBCL, we here report a patient who also had CNS involvement at initial lymphoma presentation. This case illustrates the diagnostic uncertainty in establishing the aetiology of spontaneous splenic rupture and the importance of splenic histology.