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A 54 year old man came to the emergency department with acute postprandial abdominal pain, vomiting, and collapse. He was known to have learning difficulties, chronic renal impairment, and hypertension. There were no other systemic symptoms. On examination he was apyrexial, he had a heart rate of 90 beats/min, a blood pressure of 80/50 mm Hg, and oxygen saturations of 97% on air. His chest was clear, there were no cardiac murmurs, and abdominal examination revealed a tender fullness over the right flank and hypochondrium. Lesions were noted over the second, third, and fifth toes of the left foot (fig 1).
His initial investigations showed a haemoglobin of 83 g/l, mean corpuscular volume 88.3 fl, international normalised ratio 0.95, activated partial thromboplastin time 57.9 sec (control 32.2 sec) which partially corrected to 37.8 sec with a 50/50 mix, sodium 135 mmol/l, potassium 4.6 mmol/l, urea 28.7 mmol/l, creatinine 475 μmol/l, calcium 2.34 mmol/l, magnesium 0.75 mmol/l, phosphate 1.57 mmol/l, amylase 67 IU/l, albumin 36 g/l, liver function normal, and urine, blood, and stool culture sterile. Chest and abdominal plain radiographs were unremarkable. Over the next 24 hours the haemoglobin fell to 57 g/l requiring transfusion. A gastroscopy showed grade 1 oesophagitis, a normal stomach, pylorus, and D1. A prominent ampulla at D2 was noted, showing non-specific small bowel mucosa on biopsy. A further drop in haemoglobin occurred, associated with increased abdominal pain. Faecal occult blood was negative and no external rectal bleeding was noted. Direct Coombs test was negative, reticulocyte count 27 × 109/l (50–100 × 109/l), and haptoglobins were 0.3 g/l (0.5–2.4). Repeat abdominal examination revealed a large mass in the right hypochondrium.
He had an abdominal computed tomography (fig 2). This was followed up over a six week period with serial ultrasound scans (figs 3, 4, and 5).
What is the diagnosis and what are the clinical features of this condition?
What does the computed tomogram of the abdomen (fig 2) show?
What do the renal ultrasound scans (figs 3, 4, 5) show and what is the likely diagnosis?
What is the cause of the clotting abnormality?
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