Abstract
Hyperhaemolysis is a rare but well recognized complication of transfusion in sickle cell disease, associated with increased mortality. The pathogenesis is not well understood and is likely to be multifactorial. There is no gold standard management of hyperhaemolysis; immunosuppression with steroids and intravenous immuoglobulins has been the mainstay of treatment. More recently Rituximab and Eculizumab are increasingly used. Hyperhaemolysis in pregnancy is an even more complex problem with compounded risk to the mother and foetus.
This case reports a 29 year old female with HbSS who presented with hyperhaemolysis in pregnancy at 25 weeks gestation, 7 days after a red cell transfusion for a vaso-occlusive crisis. The patient received 1 g methylprednisolone and 2g/kg immunoglobulins in divided doses. Despite this, she required further blood transfusion as the haemoglobin was declining further and was symptomatic for her anaemia. Her haemoglobin reached a nadir level of 39 g/l. Therefore she was treated with Eculizumab 900 mg iv pre transfusion to prevent exacerbation of the haemolysis triggered by the transfusion. Her haemoglobin stabilised and the haemolysis resolved within 48 hours. She delivered safely at 34 weeks gestation.
The DAT remained negative throughout and no new red cell antibodies were detected. Urine HPLC confirmed the presence of HbA and HbS in the urine, confirming the diagnosis. The urine HPLC 48 hr post Eculizumab and 2 further red cell units was clear, demonstrating no further haemolysis with the additional red cells given.
This is the first case report detailing the use of Eculizumab for the management of hyperhaemolysis in pregnancy in sickle cell disease. The patient tolerated the treatment which resulted in resolution of the haemolysis, with safe delivery at 34 weeks gestation. The possible role of complement activation in hyperhaemolysis is discussed. The use of Eculizumab in other case reports of hyperhaemolysis is reviewed. The ideal dosing schedule remains uncertain. This report strongly suggests that Eculizumab should be accessible and considered in severe cases of hyperhaemolysis which are refractory to standard treatment. It also suggests safe usage in pregnancy in sickle cell patients.
Disclosures
Scully: Novartis: Honoraria, Other: Member of Advisory Board, Speakers Bureau. Porter:Cerus: Honoraria; Novartis: Consultancy; Agios: Honoraria.
Sickle cell disease SC in pregnancy: a case report
