Anti‐IFC antibodies in a patient with CHAPLE syndrome: Implications for blood management.

In conclusion, anti-IFC antibodies are capable of inducing an acute extravascular haemolytic transfusion reaction and IFC-compatible blood should be strongly considered for elective blood transfusion in patients with CHAPLE syndrome. CHAPLE syndrome is an extremely rare autosomal recessive condition hallmarked by a complete loss of the CD55 protein, hyperactivation of complement, angiopathic thrombosis and protein-losing enteropathy based on primary lymphangiectasia.[1] The mechanism behind this syndrome is believed to reside in the fact that CD55, also known as the decay accelerating factor, mitigates complement activation and thus modulates the immune response.[[1]] Importantly, the CD55 protein harbours the entire Cromer blood group system and patients with CHAPLE syndrome characteristically produce antibodies against these antigens when exposed to red blood cells (RBCs) from CD55 SP + sp individuals.[[3], [5]] The clinical characteristics of these antibodies have not been well documented and are thus largely unclear. It is possible that anti-IFC antibodies vary between patients in their potential of causing a haemolytic transfusion reaction. [Extracted from the article]