Abstract
Evans syndrome is a rare haematological disease characterised by concurrent occurrence of autoimmune haemolytic anaemia (AIHA) and idiopathic thrombocytopenic purpura. The autoimmune haemolysis can lead to hyperbilirubinaemia, profound anaemia and haemodynamic instability. It can be difficult to treat and when refractory to medical treatment it could necessitate emergency splenectomy. Plasmapheresis is used in refractory haemolytic anaemia to aid removal of disease causing autoimmune complexes whilst immunomodulators act to reduce autoantibody response and effectiveness. However, this is an expensive and resource intensive process that is limited to tertiary referral centres. In this case study we present a successful novel alternative for life threatening AIHA in Evans Syndrome using haemofiltration with on-going fresh frozen plasma transfusion when resources are limited or it is not an accessible method.
Highlights
Autoimmune haemolytic anaemia (AIHA) is often an IgG driven condition, which is a haematological emergency requiring immunomodulation
Evans syndrome is a rare haematological disease characterised by concurrent occurrence of autoimmune haemolytic anaemia (AIHA) and idiopathic thrombocytopenic purpura
AIHA symptoms are determined by the severity of the anaemia in which intravascular red cell destruction leads to an insufficient oxygenation, cardiac failure and rise in serum bilirubin, becoming a life – threatening condition without treatment [2]
Summary
Autoimmune haemolytic anaemia (AIHA) is often an IgG driven condition, which is a haematological emergency requiring immunomodulation. The condition that combines AIHA and immune thrombocytopenia has been denominated Evans syndrome, which is more difficult to treat and has a higher mortality than AIHA presenting alone. This condition can be primary idiopathic or secondary to diverse factors: viral infections, autoimmune and connective tissue diseases, immune deficiency diseases, malignancies, babesiosis after splenectomy or hematopoietic disorders [1]. In patients failing medical management, plasmapheresis aids the removal of pathogenic immune complexes [6,7] This procedure is labour-intensive, costly and limited to specialist centres. In this case report we describe an alternative method when there is no response to medical treatment, using a conventional veno-venous haemofiltration with plasma to aid the potential removal of IgG immune complexes
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