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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
Published on: March 14, 2017
Asymptomatic child heterozygous for hemoglobin S and hemoglobin Pôrto Alegre
Liliana Lojo1, Pedro Santiago-Borrero, Enid Rivera
1Hematology Division, Department of Pediatrics, UPR School of Medicine, San Juan, Puerto Rico. lilianalojo@hotmail.com
Abstract:
Hemoglobin Pôrto Alegre (PA) is a rare hemoglobin resulting from a mutation in β9(A6)Ser → Cys. We describe an asymptomatic Puerto Rican female with combined heterozygosity for Hb PA and Hb S. Since birth, she has maintained normal hemoglobin, bilirubin, LDH levels, and reticulocyte count. Peripheral smear evaluation has revealed normal erythrocyte morphology with no changes suggestive of hemolysis. We conclude that the presence of Hb PA does not increase the risk of red blood cell sickling in patients who carry the Hb S mutation.
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