GP44 Erythropoietin as a treatment modality in hyperhaemolysis complicating sickle cell anaemia

Abstract

Introduction Hyperhaemolysis Syndrome (HS), a severe haemolytic transfusion reaction, is a rare complication in children with Sickle Cells anaemia (SCA) who require transfusion. Donor and autologous red cells are obliterated leading to a worsening of anaemia after transfusion. Erythropoietin has been reported as a treatment modality. This case series examines the experience in our tertiary Paediatric Haematology centre, of treating this haemolytic anaemia with Erythropoietin. Design and Methods Patients were identified from the SCA patient database at Our Lady’s Children’s Hospital, Crumlin, Dublin in 2018. Patient charts were reviewed Details of transfusions, presenting symptoms, examination findings, lab results and treatment modalities were recorded. Results Three children being treated with erythropoietin following HS were identified. Patient 1: Diagnosed with SCA at birth. Transfusion programme started aged 3 years 6 months due to silent infarct on MRI brain. Transfusion programme stopped aged 3 years 10 months as hyperhaemolysis suspected. Haemoglobin (Hob) fell post transfusion and HbA rose to 20% not 60–70% as would be expected. Autoantibodies were detected. This was soon complicated by Acute Splenic sequestration with associated high fever. Hob at it’s nadir 5.7 g/L. Erythropoetin commenced with good effect to date. Patient 2: SCA diagnosed aged 2 years. First transfusion aged 15 years due to crisis with Hob 6. 5 g/L. 5 weeks later admitted with abdominal pain and splenomegaly. Hob 7.45 g/L and then fell to 5.7 g/L. Treated with Immunoglobulin(IVIG) as diagnosis of HS was suspected. Hob A-13.1%. Commenced on erythropoietin and IV Iron. 2 alloantibodies detected. Hob maintained > 6 g/L since then with Erythropoetin 3 times a week and oral iron replacement. Patient 3: SCA diagnosed aged 1 year. At age 4 years, 3 admissions with chest crises and associated anaemia. Hob fell to a nadir of 4.9 g/L post transfusion and episodes were treated with IVIG and IV methylprednisolone. Another chest crisis at 4 years 7 months precipitated trial of erythropoietin for 3 months with good effect. No further transfusions required. Erythropoetin restarted aged 6 years 6 months due to Hob of 5.7 g/L in association with infection. Hob improved and patient’s Hob now maintained >6 g/L on erythropoietin three times a week. Conclusion HS is a rare complication of SCA which can cause significant worsening of anaemia that is difficult to treat. Erythropoetin can be used to maintain acceptable levels of haemoglobin to avoid transfusion.