title: Hemoglobin C associated with protection from severe malaria in the Dogon of Mali, a West African population with a low prevalence of hemoglobin S.
abstract: The malaria hypothesis proposes a survival advantage for individuals with hemoglobin variants in areas of endemic Plasmodium falciparum malaria. Hemoglobin C (HbC) is a possible example in West Africa, where this hemoglobin has a centric distribution with high frequencies among certain populations including the Dogon ethnic group. To test whether HbC is associated with protection from malaria, we performed a case-control study in the Dogon of Bandiagara, Mali. HbC was present in 68 of 391 (17.4%) of uncomplicated malaria control cases, whereas it was detected in only 3 of 67 cases (4.5%) of severe malaria (odds ratio [OR], 0.22; P =. 01). Further, HbC was present in only 1 of 34 cases (2.9%) with cerebral manifestations, the most common presentation of severe malaria in this population (OR, 0.14; P =.03). Episodes of uncomplicated malaria and parasitemias (4800-205 050/microL) were identified in cases of homozygous HbC (HbCC), which indicates that P falciparum parasites are able to efficiently replicate within HbCC erythrocytes in vivo. These findings suggest that HbC does not protect against infection or uncomplicated malaria but can protect against severe malaria in the Dogon population of Bandiagara, Mali. The data also suggest that the protective effect associated with HbC may be greater than that of HbS in this population.
authors.affiliation: National Institute of Allergy and Infectious Diseases and the National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.
authors.forename: A
authors.initials: A
authors.lastname: Agarwal
authors.forename: A
authors.initials: A
authors.lastname: Guindo
authors.forename: Y
authors.initials: Y
authors.lastname: Cissoko
authors.forename: J G
authors.initials: JG
authors.lastname: Taylor
authors.forename: D
authors.initials: D
authors.lastname: Coulibaly
authors.forename: A
authors.initials: A
authors.lastname: Koné
authors.forename: K
authors.initials: K
authors.lastname: Kayentao
authors.forename: A
authors.initials: A
authors.lastname: Djimde
authors.forename: C V
authors.initials: CV
authors.lastname: Plowe
authors.forename: O
authors.initials: O
authors.lastname: Doumbo
authors.forename: T E
authors.initials: TE
authors.lastname: Wellems
authors.forename: D
authors.initials: D
authors.lastname: Diallo
mesh_terms: D000755:Anemia, Sickle Cell / Q000097:blood / Q000453:epidemiology; D016022:Case-Control Studies; D006444:Hemoglobin C / Q000032:analysis* / Q000235:genetics; D006445:Hemoglobin C Disease / Q000097:blood / Q000453:epidemiology*; D006451:Hemoglobin, Sickle / Q000032:analysis* / Q000235:genetics; D006579:Heterozygote; D006720:Homozygote; D006801:Humans; D016778:Malaria, Falciparum / Q000097:blood* / Q000453:epidemiology* / Q000517:prevention & control; D008302:Mali / Q000453:epidemiology; D016017:Odds Ratio; D013163:Splenomegaly / Q000453:epidemiology
chemical_list: D006451:Hemoglobin, Sickle; D006444:Hemoglobin C