Erythrocytapheresis (automated red cell exchange) increasingly is used to replace sickled red cells in sickle cell disease patients as an alternative to transfusion.
Erythrocytapheresis (automated red cell exchange) increasingly is used to replace sickled red cells in sickle cell disease (SCD) patients as an alternative to transfusion.
The procedure doesn’t significantly raise the total hemoglobin — a frequent result of other methods — when replacing sickled red cells with healthy donor cells.
Erythrocytapheresis removes the patient’s sickled red cells with normal cells without significantly raising the total hemoglobin. This minimizes iron overload, which would be the case with simple transfusions where healthy red cells are transfused to the patient.
Patients can get significant iron overload in a year’s time on simple transfusion and can require chelation therapy to get rid of the excess iron, which can burdensome to the patients and families.
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No correlation was found between the duration of erythrocytapheresis and liver iron concentrations, according to a 2016 study published in the Journal of Pediatric Hematology/Oncology and co-authored by Ashok B Raj, M.D., director of the pediatric erythrocytapheresis program at the University of Louisville School of Medicine. Dr. Raj, a pediatric hematologist/oncologist, is medical director of Norton Children’s Cancer Institute, affiliated with the UofL School of Medicine.
None of 29 sickle cell disease subjects in the study maintained on long-term erythrocytapheresis had high cardiac iron concentration.
Long-term erythrocytapheresis (LTE) also can address typical lag in weight, height and growth spurt in children with SCD.
A 2014 study co-authored by Dr. Raj and published in Pediatric Blood and Cancer found peak height velocity was achieved two months earlier for females and 11 months earlier for males who started LTE before age 14. The height, weight and body mass index (BMI) of the long-term erythrocytapheresis patients were compared with that of patients with SCD from the Cooperative Study of Sickle Cell Disease.
Erythrocytapheresis typically is done on a monthly basis to help prevent complications of SCD. The treatment also can be used when a patient experiences a sickle cell crisis of severe pain, typically in the spine, arms, legs, chest or abdomen.
Sickle cell treatment available in Kentucky
Norton Children’s Cancer Institute is the largest and only pediatric health care program in Kentucky offering erythrocytapheresis for sickle cell treatment. Norton Children’s Cancer Institute offers a lifespan approach to sickle cell care, helping children with sickle cell disease transition to adult care by offering education and an individualized care plan that helps young adults feel confident to own their health care.