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KMID : 0387720120230030256
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Korean Journal of Blood Transfusion
2012 Volume.23 No. 3 p.256 ~ p.261
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A Case of Red Blood Cell Exchange Transfusion in a Patient with Hemoglobin S/b-Thalassemia
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Choi Ri-Hwa
Song Ju-Sun
Jung Hae-Kyung
Kim Se-Mi
Jung Chul-Won
Park Hyung-Doo
Ki Chang-Seok
Kang Eun-Suk
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Abstract
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Sickle cell disease and β-thalassemia are caused by abnormal hemoglobin (Hb) derived from mutation of the HBB
gene encoding β-globin. Compound heterozygous status for both mutations results in Hb S/β-thalassemia (sickle-
β-thalassemia). Vaso-occlusive phenomena and hemolysis are the clinical hallmarks and major causes of mortality.
Due to the limited availability of hematopoietic stem cell transplantation with or without gene therapy, red blood
cell (RBC) exchange transfusion is the first-line adjunctive therapy. Here we report on a successful reduction of
Hb S level in a Tunisian male sickle-β-thalassemia patient by RBC exchange transfusion for primary prophylactic
transfusion therapy before flying to his country. Results of both Ion exchange high-performance liquid chromatography
and HBB gene mutation analysis indicated sickle-β-thalassemia. Pre-erythrocytapheresis Hb S level was
80.6% of total Hb. Two volumes of RBC exchange were performed using automated erythrocytapheresis with the
COBE Spectra Apheresis System (Version 7.0, Caridian BCT, CO, USA). Post-erythrocytapheresis Hb S level was
23.4% of total Hb and hematocrit level was 32.6%, both of which met the target end points. This is the first
case report in Korea on successful RBC exchange transfusion in a patient with sickle-β-thalassemia for rapid
reduction of pathologic RBCs with Hb S.
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KEYWORD
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Sickle cell disease, Thalassemia, Blood transfusion
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