Thrombotic thrombocytopenic purpura with severe large artery branch involvement.

Letters Thrombotic thrombocytopenic purpura with severe large artery branch involvement Sir, A 36-year-old male patient was admitted to hospital due to diffuse abdominal pain, headache and blurred vision. Physical examination showed a patient with an affected clinical condition but awake. Arterial blood pressure (BP) was 260/140 mmHg with a regular heart rhythm. Abdominal examination revealed severe mesogastrium tenderness with preserved bowel sounds, and neurological examination showed no focal abnormalities. Fundoscopy showed diffuse haemorrhagic exudates without papilloedema. Laboratory investigations at admission showed thrombocytopenia (plate-lets 52 Â 10 9 /l), haemolytic anaemia [haemoglobin 10 g/dl, 2–3 schistocytes, haptoglobin 23 mg/dl, lactate dehydrogen-ase (LDH) 37 mkat/l], direct Coombs test negative and acute renal failure (creatinine 228 mmol/l) with proteinuria (þþ) and haematuria (þþþ). Immunological studies were negative for antinuclear antibodies, anti-double-stranded DNA, anti-phospholipid antibodies and anti-Scl-70. Serologies for Epstein–Barr virus, hepatitis B and C virus as well as human immunodeficiency virus (HIV) were negative. Despite an adequate arterial blood pressure control with antihypertensive drugs (BP 120/80 mmHg), the patient developed progressive confusion, and haemolytic anaemia and renal failure got worse at the same time. These findings led to the diagnosis of thrombotic thrombocytopenic pur-pura (TTP)/haemolytic uraemic syndrome (HUS), so plasma exchange (3 l per session) and haemodialysis were performed without achieving good results. The patient deteriorated; he developed an acute pancreatitis, which led to pancreatico-duodenectomy with subtotal antrectomy and cholecystec-tomy being performed. After surgery, there was an improvement of haemolysis (haptoglobin 168 mg/dl, LDH 13 mkat/l, 2–3 schistocytes, platelets 94 Â 10 9 /l) without recovery of renal failure (creatinine 632 mmol/l). The patient remained in a persistent coma, and a new brain computed tomography (CT) scan showed multiple ischaemic lesions in both cerebral hemispheres. Cerebral angiography showed occlusion of multiple large artery branches. On the 15th post-operative day, the patient died due to persistent ventricular arrhythmias. The autopsy study showed thrombotic microangiopathy of small, medium and large artery branches, with extensive haemorrhagic infarction in the brain and pancreas. In the kidney, there was also intimal hyperplasia in the arterioles and middle size arteries (Figure 1). On the other hand, in the heart, there was an area of myocardial necrosis with coronary atheromatous disease involving two large arteries but without microangiopathic lesions. TTP/HUS is a multisystemic disease characterized by fever, thrombocytopenia, microangiopatic haemolytic anaemia, neurological disorders and renal failure [1]. In the majority of clinical reports of TTP/HUS, the vascular lesions are characterized …