Alkali-resistant Hemoglobin in Sickle Cell Disease

Article1 May 1966Alkali-resistant Hemoglobin in Sickle Cell DiseaseJOHN N. BICKERS, M.D.JOHN N. BICKERS, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-64-5-1028 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptA substantial percentage of patients with homozygous hemoglobin S disease produce detectable amounts of alkali-resistant hemoglobin* during their adult life. The alkali-resistant hemoglobin lacks the pathologic physical properties of hemoglobin S; thus, the capacity to produce alkali-resistant hemoglobin is of potential importance as a source of functionally normal hemoglobin in sickle cell disease. The recognition of factors that affect alkali-resistant hemoglobin production may provide insight into the basic mechanisms that control its synthesis.The literature contains remarkably little information on the natural history of alkali-resistant hemoglobin in sickle cell disease and even less concerning the variables that affect its production....References1. SINGERCHERNOFFSINGER HAJL: Studies on abnormal hemoglobin. I. Their demonstration in sickle cell anemia and other hematologic disorders by means of alkali denaturation. Blood 6: 413, 1951. CrossrefMedlineGoogle Scholar2. BEAVENELLISWHITE GHMJJC: Studies on human foetal haemoglobin. III. The hereditary haemoglobinopthies and thalassaemias. Brit. J. Haemat. 7: 169, 1961. CrossrefMedlineGoogle Scholar3. JACKSONODOMBELL JFJLWM: Amelioration of sickle cell disease by persistent fetal hemoglobin. JAMA 177: 867, 1961. CrossrefMedlineGoogle Scholar4. ALLENJANDL DWJH: Factors influencing relative rates of synthesis of adult and fetal hemoglobin in vitro. J. Clin. Invest. 39: 1107, 1960. CrossrefMedlineGoogle Scholar5. RUCKNAGELCHERNOFF DLAI: Immunologic studies of hemoglobins. III. Fetal hemoglobin changes in the circulation of pregnant women. Blood 10: 1092, 1955. CrossrefMedlineGoogle Scholar6. RUCKNAGEL DL: Problems of Cooley's anemia. Panel discussion. Ann. N. Y. Acad. Sci. 119: 812, 1964. Google Scholar7. REEDBRADLEYRANNEY LJTBHM: The effect of amelioration of anemia on the synthesis of fetal hemoglobin in sickle cell anemia. Blood 25: 37, 1965. CrossrefMedlineGoogle Scholar8. SMITHIES O: Zone electrophoresis in starch gels: group variations in the serum proteins of normal human adults. Biochem. J. 61: 629, 1955. CrossrefMedlineGoogle Scholar9. ROBINSONROBSONHARRISONZUELZER ARMAPWW: A new technique for differentiation of hemoglobin. J. Lab. Clin. Med. 50: 745, 1957. MedlineGoogle Scholar10. ZONZISHUISMAN JHTH: A Laboratory Manual of Abnormal Hemoglobins. Charles C Thomas, Publisher, Springfield, Ill., 1958. Google Scholar11. YAKULISHELLER VJP: An elution test for the visualization of hemoglobin S in blood smears. Blood 24: 198, 1964. CrossrefMedlineGoogle Scholar12. CONLEYWEATHERALLRICHARDSONSHEPARDCHARACHE CLDJSNMKS: Hereditary persistence of fetal hemoglobin: a study of 79 affected persons in 15 Negro families in Baltimore. Blood 21: 261, 1963. CrossrefMedlineGoogle Scholar13. ZUELZERROBINSONBOOKER WWARCR: Reciprocal relationship of hemoglobins A2 and F in beta-thalassemias, a key to the genetic control of hemoglobin F. Blood 17: 393, 1961. CrossrefMedlineGoogle Scholar14. CHARACHECONLEY SCL: Rate of sickling of red cells during deoxygenation of blood from persons with various sickling disorders. Blood 24: 25, 1964. CrossrefMedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: New Orleans, LouisianaFrom the Department of Medicine, Louisiana State University School of Medicine, New Orleans, La.This study was supported by a grant from the Cancer Association of Greater New Orleans, Inc., and by grant FR 5376 to the Louisiana State University from the National Institutes of Health, Bethesda, Md.Requests for reprints should be addressed to John N. Bickers, M.D., 1542 Tulane Ave., New Orleans, La. 70012.*The designation "alkali-resistant hemoglobin" is used in preference to fetal hemoglobin or hemoglobin F, although they are commonly used interchangeably. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byFetal hemoglobin and the irreversibly sickled cell in sickle cell diseasePregnancy in sickle-cell anemia in a Caucasian groupCholelithiasis in sickle cell anemia in a caucasian populationFetal and embryonic haemoglobins.Chemical heterogeneity of fetal hemoglobin in subjects with sickle cell anemia, homozygous hb-c disease, sc-disease, and various combinations of hemoglobin variantsHomozygous sickle cell disease with coexistent hereditary spherocytosis in three siblingsNormal and Abnormal Human HemoglobinsInherited disorders of hemoglobin synthesis and pregnancyHemoglobinopathic dysfunction of the red cell 1 May 1966Volume 64, Issue 5Page: 1028-1033KeywordsBiostatisticsHemoglobinHormonesSickle cell disease ePublished: 1 December 2008 Issue Published: 1 May 1966 PDF downloadLoading ...