If both hemoglobin A and hemoglobin S
have the same oxygen-carrying capacity
and exhibit the same oxygen saturation
curves when at low hemoglobin concentrations, then why does hemoglobin S
have a decreased oxygen-carrying capacity at the concentrations found in the red
cell? Trace the path of a red cell in sickle
cell disease from the lungs to the capillary
bed and back to the lungs, noting the hemoglobin changes that are likely to occur
during the journey.
Bain BJ: Haemoglobinopathy Diagnosis. Blackwell Science, Oxford, UK, 2001.
Claster S, Vichinsky EP: Managing sickle cell disease.Br Med J 327:1151–1155, 2003.
De Franceschi L, Corrocher R: Established and experimental treatments for sickle cell disease.
Haematologica 89:348–356, 2004.
Herrick JB: Peculiar elongated and sickle shaped
red blood corpuscles in a case of severe anemia.
Arch Intern Med 6:517–521, 1910.
Jison ML, Munson PJ, Barb JJ, et al.: Blood mononuclear cell gene expression profiles characterize
the oxidant, hemolytic and inflammatory stress
of sickle cell disease.Blood 104:270–280, 2004.
Josephs R: Research on sickle cell hemoglobin, virtual tour of sickle hemoglobin polymerization.
Laboratory for Electron Microscopy at the University of Chicago, 1999. Available at: http://
Lenfant C, National Institutes of Health, National
Heart Lung and Blood Institute: The Management of Sickle Cell Disease. 2002. Publication
No. 02-2117. Available at: www.nhlbi.nih.gov/
Locatelli F, Stefano PD: New insights into
haematopoietic stem cell transplantation for patients with haemoglobinopathies. Br J Haematol 125:3–11, 2004.
Manci EA, Culberson DE,Yang YM, et al., and Investigators of the Cooperative Study of Sickle Cell
Disease:Causes of death in sickle cell disease:an
autopsy study. Br J Haematol 123:359–365,
Mason VR: Sickle cell anemia.JAMA 79:1318–1320;
Neel JV:The inheritance of sickle cell anemia. Science 110:64–65, 1949.
Old JM: Screening and genetic diagnosis of haemoglobin disorders.Blood Rev 17:43–53, 2003.
Roberts I: The role of hydroxyurea in sickle cell
disease.Br J Haematol. 120:177–186, 2003.
Serjeant GR:The emerging understanding of sickle
cell disease.Br J Haematol 112:3–18, 2001.
Serjeant GR, Serjeant BE: Sickle Cell Disease. 3rd
ed. Oxford University Press, New York, 2001.
Steinberg MH, Forget BG, Higgs DR, et al.: Disorders of Hemoglobin. Cambridge University
Press, Cambridge, UK, 2001.
Vichinsky EP, Neumayr LD, Earles AN, et al.:
Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute
Chest Syndrome Study Group. N Engl J Med
Walters MC, Storb R, Patience M, et al.: Impact of
bone marrow transplantation for symptomatic
sickle cell disease: an interim report. Multicenter investigation of bone marrow transplantation for sickle cell disease. Blood 95:
Weatherall DJ:Towards molecular medicine: reminiscences of the haemoglobin field, 1960–2000.
Br J Haematol. 115:729–738, 2001.