总结

• Haemolytic anaemia characterised by evidence of intravascular haemolysis such as haemoglobinuria and elevation of plasma lactate dehydrogenase.
• Diagnosed by flow cytometry of granulocytes and red blood cells, looking for the lack of the glycosylphosphatidylinositol anchor or of proteins dependent on it for attachment to the cell membrane.
• Complicated by thrombosis, particularly of hepatic and other intra-abdominal veins, cerebral veins, and other usual and unusual venous sites.
• Specifically treated with a monoclonal antibody to the fifth component of complement (eculizumab).
• Related to aplastic anaemia in that it may arise in patients with or recovering from aplastic anaemia, and it may manifest with cytopoenias that are the result of marrow hypoproduction, which may respond to treatment for aplastic anaemia.