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104 Disorders of Hemoglobin
Edward J. Benz, Jr.

	Figure 104-1  The globin genes. The α-like genes (α,ζ) are encoded on chromosome 16; the β-like genes (β,γ,δ,ε) are encoded on chromosome 11. The ζ and ε genes encode embryonic globins.
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	Figure 104-2  Hemoglobin-oxygen dissociation curve. The hemoglobin tetramer can bind up to four molecules of oxygen in the iron-containing sites of the heme molecules. As oxygen is bound, 2,3-BPG and CO2 are expelled. Salt bridges are broken, and each of the globin molecules changes its conformation to facilitate oxygen binding....
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	Figure 104-3  Pathophysiology of sickle cell crisis.
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	Figure 104-4  Sickle cell anemia. The elongated and crescent-shaped red blood cells seen on this smear represent circulating irreversibly sickled cells. Target cells and a nucleated red blood cell are also seen.
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	Figure 104-5  β-Thalassemia intermedia. Microcytic and hypochromic red blood cells are seen that resemble the red blood cells of severe iron deficiency anemia. Many elliptical and teardrop-shaped red blood cells are noted.
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