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About the Authors:
Michael A. Krause
Affiliation: Laboratory of Malaria and Vector Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland, United States of America
Seidina A. S. Diakite
Affiliation: Malaria Research and Training Center, Faculty of Medicine, Pharmacy, and Odontostomatology, University of Bamako, Bamako, Mali
Tatiana M. Lopera-Mesa
Affiliation: Laboratory of Malaria and Vector Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland, United States of America
Chanaki Amaratunga
Affiliation: Laboratory of Malaria and Vector Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland, United States of America
Takayuki Arie
Affiliation: Department of Physics and Electronics, School of Engineering, Osaka Prefecture University, Osaka, Japan
Karim Traore
Affiliation: Malaria Research and Training Center, Faculty of Medicine, Pharmacy, and Odontostomatology, University of Bamako, Bamako, Mali
Saibou Doumbia
Affiliation: Malaria Research and Training Center, Faculty of Medicine, Pharmacy, and Odontostomatology, University of Bamako, Bamako, Mali
Drissa Konate
Affiliation: Malaria Research and Training Center, Faculty of Medicine, Pharmacy, and Odontostomatology, University of Bamako, Bamako, Mali
Jeffrey R. Keefer
Affiliation: Division of Pediatric Hematology, Department of Pediatrics, Johns Hopkins School of Medicine, Baltimore, Maryland, United States of America
Mahamadou Diakite
Affiliation: Malaria Research and Training Center, Faculty of Medicine, Pharmacy, and Odontostomatology, University of Bamako, Bamako, Mali
Rick M. Fairhurst
* E-mail: [email protected]
Affiliation: Laboratory of Malaria and Vector Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland, United States of America
Introduction
α-thalassemia is an inherited disorder of hemoglobin (Hb) synthesis, in which reduced production of α-globin chains leads to decreased amounts of normal α2β2 tetramers and increased amounts of unpaired β-globin chains. In sub-Saharan Africa, α-thalassemia states are produced by a 3.7-kb deletion that leaves one functional copy of duplicated α-globin genes. Heterozygotes (−α/αα) have an essentially normal phenotype while homozygotes (−α/−α) have mild microcytic anemia. HbH disease is a chronic hemolytic disorder that may produce severe anemia requiring periodic blood transfusions. While the prevalence of α-thalassemia can exceed 50% in some malaria-endemic areas of sub-Saharan Africa, HbH disease (−/−α) is extremely rare because the mutant cis allele (−/) is very uncommon.
One study associated α-thalassemia with reduced risk of severe Plasmodium falciparum malaria in Papua New...