First successful automated red cell exchange (erythrocytapheresis) in Nigeria for a sickle cell anaemia patient with priapism: A case report

Abstract

INTRODUCTION: Despite several documented challenges, Hematopoietic Stem cell Transplantation (HSCT) remains the only curative therapy for Sickle Cell Disease (SCD). However HSCT is expensive with its complications, therefore supportive care remains the main available option to many Nigerians. Frequent blood transfusion carries significant complications especially iron overload. Red cell exchange offers a better control both as prophylaxis and therapy for some complications.
METHOD: A 32years old male SCD patient had priapism as an indication for Red Cell exchange. A total of 1850ml of his red cells was exchanged with fresh eight units of red cell concentrates, group identical (ABO and RhD), Hemoglobin phenotype AA and screened for routine transfusion transmissible infections. A continuous apheresis machine (COBE SPECTRA VERSION 7.1) was used and the procedure lasted for 195 minutes.
RESULTS: We report a successful automated Red Cell Exchange for SCD. Final fluid balance was 90% with only 19% of fraction of patient’s red cells remaining. A positive Hematocrit balance was achieved from his usual stable Hematocrit of 26% to 32% after the exchange. Also irreversible sickle cells were reduced from 10% before to <1% post apheresis. Patient tolerated the procedure except for mild chills and rigor. Post procedure evaluation within 24hours showed Hematocrit of 27.7% and priapism had completely resolved. He was discharged home after 48hrs for follow up.
CONCLUSION: HSCT is curative for SCD but expensive. However frequent supportive Blood transfusion which is the current practice in Nigeria has several complications. Red cell exchange has been shown to be a more effective supportive care for SCD but challenges of blood donation will be of great concern if this procedure becomes routine in the future.

Key words: Sickle cell disease, Red Cell Exchange, Apheresis.