Abstract Ref Number = APCP785
Coombs Positive Haemolytic Anaemia complicating recrudescent Kawasaki Disease patients receiving high dose intravenous immunoglobulin
Liang Choo Hung,Mathan Munusamy,Sanjay Woodhull,Kok Kuan Choo
Institut Pediatrik Hospital Kuala Lumpur Malaysia Subang Jaya Medical Centre, Ramsay Sime Darby Healthcare Malaysia
Background : Kawasaki disease (KD) is an acute vasculitis of childhood that can lead to coronary artery aneurysms. The primary treatment of acute illness is intravenous immunoglobulin (IVIG) 2g/kg, together with aspirin within the first 10 days of fever.
Case Presentation Summary : We describe 3 patients who developed haemolytic anaemia following treatment with IVIG for KD, the 1st such report in Malaysia. The first patient, a 10 month old girl, presented with classical recrudescent KD which was treated with aspirin and two doses of IVIG. She developed marked haemolytic anaemia following the second IVIG with positive direct Coombs test. She responded to blood transfusion and methylprednisolone. The second patient is a 22 month old girl, whom presented as above and was given 2 doses of IVIG. She subsequently developed mild Coombs positive haemolysis, treated successfully with prednisolone. The last patient, a 3 years 9 month old boy, with classical KD, was refractory to two courses of IVIG and needed methylprednisolone. He developed Coombs positive haemolysis requiring blood transfusion and prednisolone. Haemolytic anaemia in KD patients receiving IVIG is multifactorial in origin. All 3 of our patients developed haemolysis after receiving the 2nd dose of IVIG, indicating that the haemolysis is probably a dose dependent antibody mediated response. The negative complement and elution tests indicate that the offending antibodies were from IVIG and not autoantibodies. Non-O blood group patients tend to develop greater haemolysis and concurringly, significant haemolysis occurred in the two patients who were AB Rh positive. This could be explained by the presence of both anti-A and anti-B isohemagglutinins in IVIG resulting in greater hemolysis in patients expressing both A and B antigens.
Learning Points/Discussion : Our findings indicate that recrudescent KD patients who receive high dose IVIG are at higher risk of developing immune mediated haemolysis and awareness of this complication is important.
Keywords: Kawasaki Disease Intravenous immunoglobulin Haemolytic anaemia Coombs positive