Prebypass Pheresis and Red Blood Cell Exchange in a Patient with Homozygous SS Sickle Cell Disease Undergoing Cardiopulmonary Bypass: A Case Report

Abstract

Sickle cell disease was first described by Herrick in 1910. This disease involves an abnormality of the hemoglobin molecule which, under certain conditions, causes the red cell to take on a sickle shape. This abnormal shape and hemoglobin prevent the red cell from performing the normal respiratory functions and interfere with the normal flow through the circulatory system. Individuals demonstrate either a homozygous (dominant) SS or a heterozygous Ss state. Clinical symptoms for patients with SS disease are most often characterized by recurrent painful crises, sequestration of sickled cells, chronic hemolytic anemia, systemic diseases, vasoocclusive crises, permanent organ damage and recurrent infection. These patients usually require exchange transfusions due to the concern that the stress of surgery will precipitate a sickling crisis. Exchange transfusions require large amounts of homologous red blood cells (RBCs) to lower the percentage of the homozygous SS hemoglobin. The use of autologous pheresis in the peri operative period reduces the amount of homologous RBCs required and lowers the patient's exposure to homologous blood and its associated risks. This paper discusses the disease, the planning and the techniques for prebypass pheresis and red cell exchange in a patient with homozygous SS hemoglobin sickle cell disease undergoing cardiopulmonary bypass.