Red blood cell exchange. First report in a Pediatric Hospital in Quito-Ecuador

Abstract

Introduction: Sickle-cell anemia is the most common structural hemoglobinopathy worldwide and is caused by the production of hemoglobin S (HbS) as a result of a point mutation in the beta globin gene.

Objective: Show the benefits of red blood cell replacement by apheresis in the presentation of acute chest syndrome, caused by sickle-cell anemia.

Case Presentation: We describe the management of a thirteen-year-old patient with sickle-cell anemia, who presented, at admission to the hospital, an occlusive vessel crisis, secondary to her underlying pathology. On the second day of admission, she presented acute chest syndrome. Erythrocyte replacement was requested to the hospital blood service. After analyzing the case, the total blood volume of the patient was calculated, red blood cell concentrates (RBCs) compatible with the patient were prepared, all RBCs were filtered, and the hematocrit was quantified in all RBCs. The procedure was performed with the apheresis equipment COM.TEC.  in which a total of  1,200 milliliters of erythrocytes was replaced.

Discussion: The erythrocyte replacement by apheresis contributed a remarkable and  visible  clinical  and laboratory improvement. In our view, we consider that the procedure was efficient.

Conclusions: The replacement of erythrocytes by apheresis in the acute thoracic syndrome in sickle cell crisis is a procedure that may be used in patients who do not respond to other therapies, benefiting from minimal alteration of the viscosity and blood volume in the patient, as well as concomitant decrease of hemoglobin S concentration.

Key words: Erythropheresis, acute thoracic syndrome, sickle-cell disease.