Complications
Develops in 2% to 5% of patients with PNH, usually the acute myelogenous type.[18] The diagnosis is made by inspection of the blood or bone marrow biopsy. This normally does not respond well to usual treatment.
About 10% of patients with PNH have hepatic vein thrombosis. It is especially likely during pregnancy or with severe haemoglobinuric episodes. It is best diagnosed by computed tomography or flow Doppler of the hepatic veins.[61] It is treated with thrombolysis (if recent), anticoagulation (low-molecular-weight heparin by preference), and eculizumab, which seems to prevent its occurrence.[61]
Other thromboses occur with high frequency in PNH. They are diagnosed and treated in much the same way as the Budd-Chiari syndrome: thrombolysis (if recent), anticoagulation (low-molecular-weight heparin by preference), and eculizumab, which seems to prevent its occurrence.[61]
Probably the second most common type of thrombosis (after abdominal events).[20]
Venous sinus thrombosis is more common. Arterial events also occur. Magnetic resonance imaging and magnetic resonance angiography are the best methods for diagnosis. Treatment is mainly supportive. There is no demonstrated benefit for anti-oedema and anticoagulation therapy.[62]
Probably 40% of patients have significant renal dysfunction, and 5% to 15% die of renal failure.[18] The cause of death is presumably damage from haemoglobinuria. Treatment, once renal failure has occurred, is as for any other renal failure (dietary changes, phosphate-lowering measures, renal dialysis, etc). Eculizumab may prevent renal failure.
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