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The British Journal of Radiology, 77 (2004), 876877 DOI: 10.

1259/bjr/17267873

2004 The British Institute of Radiology

Case report

Polycythaemia vera presenting as massive splenic infarction and liquefaction


D BECKETT, MBBS, C MILLER, MBBS, J R FERRANDO, MB, FRCR and A K BANERJEE, MBBS, FRCP, FRCR
Birmingham Heartlands Hospital, Birmingham Heartlands and Solihull Hospitals NHS Trust, Bordesley Green East, Birmingham B9 5SS, UK

Thrombotic events in polycythaemia vera (PV) are common [1]. The typical appearances of a splenic infarct as a low attenuation, peripheral, wedge-shaped area are well described [2]. Massive splenic infarction and liquefaction in PV is rare. Only one previously documented case report exists in a patient with a 2 year history of PV [3]. We present a case of splenic infarction with massive liquefaction as a rst presenting feature of PV. Multislice contrast enhanced CT scanning allows the imaging of the thrombus itself. The CT features are illustrated and the important clinical and radiological differential of a splenic abscess is discussed.

Discussion
PV is a condition characterized as a panhyperplastic, malignant marrow disorder. The most prominent feature is the rise in red cell mass, often accompanied by rises in

Case report
A 51-year-old woman presented with a 4 month history of recurrent abdominal pain localized to the left hypochondrium, in association with mild pyrexia, leukocytosis (white all count 186109 l21), thrombocytosis (platelets 7096109 l21) and a haemoglobin of 19.5 g dl21. 4 months prior to this admission she was admitted with a history suspicious of pulmonary embolism. Following a negative CT pulmonary angiogram she was discharged. An initial abdominal radiograph was unremarkable. Abdominal ultrasound showed a normal appearance of the liver and kidneys with mild splenomegaly (18 cm). Due to the recurrent abdominal pain, an abdominal CT scan was performed. The scan was conducted on a Toshiba Asteion multislice system (Toshiba, Tokyo, Japan) with oral and intravenous contrast (100 ml of Niopam 300) using a pitch of 5.5 and a 3 mm image slice thickness. The CT scan (Figures 1 and 2) showed a complex intrasplenic uid collection in association with a splenic vein thrombosis. No evidence of pancreatitis or diverticulitis as a source of mesenteric emboli was evident. The portal vein was patent and no gas was seen within the infarcted spleen. A diagnosis of PV was conrmed with associated splenic vein thrombosis and splenic infarction with liquefaction. Following diagnosis she was commenced on regular anticoagulation, venesection, hydroxyurea and allopurinol and currently remains well.
Received 15 August 2003 and in revised form 5 December 2003, accepted 3 February 2004. Address correspondence to Dr A K Banerjee.

Figure 1. Contrast enhanced CT. A thrombus is seen as an intraluminal lling defect within the splenic vein (arrowhead).

Figure 2. Contrast enhanced view demonstrating intrasplenic


liquefaction. Note the absence of gas within the collection. Arrowheads indicate the spleen.

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The British Journal of Radiology, October 2004

Case report: Polycythaemia vera

white blood cell and platelet counts. Leukaemic conversion occurs in 13% and the median survival with treatment is 13 years [4]. Untreated life expectancy is approximately 18 months from diagnosis [4, 5]. The presenting symptoms are related to hyperviscosity and the associated splenomegaly. Thrombotic complications are common, with up to 20% of patients having thrombotic events as their presenting symptom [1, 4]. It has been estimated that 14% of patients have thrombotic events preceding diagnosis [1]. CT is a sensitive method for the diagnosis of abdominal venous thrombus [6]. On postcontrast CT, the thrombus is seen typically as a mixed or low attenuation, intraluminal lling defect. Venous thrombosis maybe identied on a pre-contrast scan. Typical features are of a hyperdense intraluminal defect in the acute presentation. The density of the thrombus decreases as clot lysis takes place, at which point the thrombus is isodense to blood. Multislice CT allows accurate identication of venous thrombus with minimal motion artefact. In this case the CT diagnosis was relatively clear. An intrasplenic uid collection with no gas and an associated splenic vein thrombosis conrms the diagnosis of massive splenic infarction and liquefaction secondary to splenic vein thrombosis. In the absence of visualization of the splenic thrombus these imaging ndings with non-specic clinical features of splenic vein thrombosis may suggest the presence of a splenic abscess [79]. In this situation the presence of gas may be an important radiological sign, distinguishing a splenic abscess from an infarction. Splenic infarction with gas formation, however, has been described [10]. No denitive criteria exists for the differentiation between splenic infarction with liquefaction and a splenic abscess [3]. While the management of a splenic abscess requires splenectomy or percutaneous drainage, that of splenic infarction with liquefaction requires no intervention, except in the management of the primary cause. In our case the splenic infarction was due to the patients polycythaemia. Massive splenic infarction may also be

seen in sickle cell disease, portal hypertension, pancreatitis, malignancy and infection. This case illustrates the CT ndings of splenic infarction with liquefaction in a patient presenting with PV and highlights the potential difculties that may exist in the differentiation between splenic infarction with liquefaction and a splenic abscess.

References
1. Gruppo Italiano Studio Policitemia. Polycythemia vera: the natural history of 1213 patients followed over 20 years. Ann Intern Med 1995;123:65664. 2. Belli AM, Jennings CM, Nakielny RA. Splenic and portal venous thrombosis: a vascular complication of pancreatic disease demonstrated on computed tomography. Clin Radiol 1990;41:136. 3. Downer W, Peterson S. Massive splenic infarction and liquefactive necrosis complicating polycythemia vera. AJR Am J Roentgenol 1993;161:7980. 4. Gumina RJ, Foley DA, Tefferi A. Polycythemia veraa case report and discussion on pathogenic mechanisms of increased thrombosis. Angiology 2002;53:58791. 5. Rosenthal N, Bassen FA. Course of polycythemia. Arch Intern Med 1938;62:90317. 6. Gayer G, Zandman-Goddard G, Raanani P, Hertz M, Apter S. Widespread abdominal venous thrombosis in paroxysmal nocturnal hemoglobinuria diagnosed on CT. Abdom Imaging 2001;26:4149. 7. OKeefe JH Jr, Holmes DR, Schaff HV. Thromboembolic splenic infarction. Mayo Clin Proc 1986;61:96772. 8. Van der Lann RT, Verbeeten B, Smits NJ, Lubbers MJ. Computed tomography in the diagnosis and treatment of solitary splenic abscesses. J Comput Assist Tomogr 1989;13: 714. 9. Nores M, Phillips EH, Morgenstern L, Hiatt JR. The clinical spectrum of splenic infarction. Ann Surg 1998;64:1828. 10. Levy JM, Wasserman PI, Weiland DE. Non suppurative gas formation in the spleen after transcatheter splenic infarction. Radiology 1981;139:3756.

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