Hemoglobin S-D Disease
Authors: M. J. CAWEIN
Journal: Annals of Internal Medicine (1966)

Article1 January 1966Hemoglobin S-D DiseaseM. J. CAWEIN, M.D., E. J. LAPPAT, M.D., R. W. BRANGLE, M.D., C. H. FARLEY, M.D.M. J. CAWEIN, M.D., E. J. LAPPAT, M.D., R. W. BRANGLE, M.D., C. H. FARLEY, M.D.Author, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-64-1-62 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptItano (1) first described hemoglobin D in 1951 in a Caucasian family who had hemoglobin S interacting with D. Hemoglobin D was the fifth hemoglobin to be described. To date, 11 cases of hemoglobin S-D disease have been reported in the literature. It is the purpose of this paper to report four additional cases of hemoglobin S-D disease in unrelated Negro families. Since patients with hemoglobin S-D disease present with anemia, circulating sickle cells in the venous blood, and a hemoglobin that migrates with hemoglobin S on paper electrophoresis at pH 8.6, they are frequently diagnosed as sickle cell anemia....References1. ITANO HA: Third abnormal hemoglobin associated with hereditary hemolytic anemia. Proc. Nat. Acad. Sci. 37: 775, 1951. CrossrefMedlineGoogle Scholar2. CARTWRIGHT GW: Diagnostic Laboratory Hematology, 2nd ed. Grune & Stratton, New York, 1958. Google Scholar3. BRANGLECAWEINLAPPAT RWMJEJ: A new rapid method for agar gel electrophoresis. Amer. J. Clin. Path. 43: 497, 1965. CrossrefGoogle Scholar4. MARKS PA: Enzymes of the pentose phosphate pathway. Meth. Med. Res. 9: 24, 1961. MedlineGoogle Scholar5. CHARACHECONLEY SCL: Rate of sickling of red cells during deoxygenation of blood from persons with various sickling disorders. Blood 24: 25, 1964. CrossrefMedlineGoogle Scholar6. SMITHCONLEY EWCL: Clinical features of the genetic variants of sickle cell disease. Bull. Hopkins Hosp. 94: 289, 1954. MedlineGoogle Scholar7. SINGERSINGERGOLDBERG KLSR: Studies on abnormal hemoglobins. XI. Sickle cell-thalassemia disease in the Negro. The significance of S+ A + F and S + A patterns obtained by hemoglobin analysis. Blood 10: 405, 1955. CrossrefMedlineGoogle Scholar8. STURGEONITANOBERGREN PHAWR: Clinical manifestations of inherited abnormal hemoglobins. I. Interaction of hemoglobin-S with hemoglobin-D. Ibid., p. 389. Google Scholar9. DACIE JW: The Hemolytic Anemias, Part I, The Congenital Anemias, 2nd ed. Grune & Stratton, New York, 1960, p. 270. Google Scholar10. SMITHCONLEY EWCL: Sickle cell-hemoglobin D disease. Ann. Intern. Med. 50: 94, 1959. LinkGoogle Scholar11. STEWARTMACIVER JWJE: Sickle cell hemoglobin D disease in a mulatto girl. Lancet 1: 23, 1956. CrossrefGoogle Scholar12. ARENDSLAYRISSEROMERO TMRA: Sickle cell-hemoglobin D disease in a Portuguese child. Acta Haemat. 22: 118, 1959. CrossrefMedlineGoogle Scholar13. MCCURDY PR: Clinical and physiologic studies in a Negro with sickle-cell hemoglobin D disease. New Eng.. J. Med. 262: 961, 1960. CrossrefGoogle Scholar14. PINEAU A: Premier cas en Guadeloupe d'anemie hemolytique genotypique avec drepanocytose et hemoglobinose D histoire d'un diagnostic. Bull. Soc. Path. Exot. 56: 485, 1963. Google Scholar15. FIGUEROAARENDS ET: Enfermedad por drenapanocitosis y hemoglobina D complicada con anemia megaloblastica en un nino Venezolano. Arch. Venez. Pueric. 26: 494, 1963. Google Scholar16. WATSON-WILLIAMSBEALEIRVINELEHMANN EJDDH: A new hemoglobin, D Ibadan (β-87 threonine to lysine) producing no sickle-cell hemoglobin D disease with hemoglobin S. Nature (London) 205: 1273, 1965. CrossrefMedlineGoogle Scholar17. RESTREPOLONDONO AMOG: Sickle cell hemoglobin D disease in a Negro Colombian patient. Arch. Intern. Med. 62: 1301, 1965. AbstractGoogle Scholar18. BENZERINGRAMLEHMANN SVMH: Three varieties of human haemoglobin D. Nature (London) 182: 852, 1958. CrossrefMedlineGoogle Scholar19. BAGLIONI C: Abnormal human haemoglobins VII. Chemical studies on haemoglobin D. Biochem. Biophys. Acta 59: 437, 1962. CrossrefMedlineGoogle Scholar20. BABINJONESSCHROEDER DRRTWA: Hemoglobin D—Los Angeles: alpha-2A beta-2-121 gluNH2. Biochim. Biophys. Acta 86: 136, 1964. CrossrefMedlineGoogle Scholar21. Murayama M: A molecular mechanism of sickled erythrocyte formation. Nature (London) 202: 258, 1964. CrossrefMedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAuthors: M. J. CAWEIN, M.D.; E. J. LAPPAT, M.D.; R. W. BRANGLE, M.D.; C. H. FARLEY, M.D.Affiliations: Lexington, KentuckyFrom the Departments of Medicine and Pathology, University of Kentucky Medical Center, Lexington, Ky.This study was supported in part by grant AM 073602 and FR 0537-01, the U. S. Public Health Service, Washington, D. C.This study was presented in part at the regional meeting of the American College of Physicians, Louisville, Ky., September, 1964.Requests for reprints should be addressed to M. J. Cawein, M.D., Department of Medicine, University of Kentucky Medical Center, Lexington, Ky. 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