Recurrent cerebral ischaemic events in the setting of paroxysmal nocturnal haemoglobinuria

Abstract

The authors present a case of a 28-year-old man with a known history of paroxysmal nocturnal haemoglobinuria (PNH), under platelet antiaggregation, admitted following recurrent transitory arterial ischaemic attacks. Concomitant thrombosis of the superior mesenteric artery and bilateral renal infarction was found. Cardioembolism, namely patent foramen ovale, was excluded and anticoagulation added, with no further events on 10-month follow-up. PNH is a rare acquired disorder of haematopoietic stem cells, characterised by haemolytic anaemia, pancytopenia and thrombotic events classically involving the venous system. Reports of cerebral artery stroke and accompanying intra-abdominal arterial thrombosis are especially rare. Complementary investigation and treatment options in these patients are discussed.

Figure 1

First diffusion-weighted brain MRI displays acute infarctions in multiple topographies. (A) Left frontal convexity cortex; (B) Right parietal convexity cortex; (C) Left cerebellar hemisphere.

Figure 2

Abdominopelvic CT with contrast ((A) arterial phase; (B) portal venous phase). Heterogeneous contrast enhancement of the hepatic parenchyma in the arterial phase, which homogenise in the portal phase, suggestive of non-specific early changes in vascularisation; multiple wedge-shaped hypovascularised areas in both kidneys, observed in acquisition phases and indicative of infarction.

Figure 3

Second diffusion-weighted brain MRI. (A) Left fronto-opercular cortex acute infarction; (B) Right parietal medium convexity cortex acute infarction.