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Hereditary Elliptocytosis

• Silent carrier

• Mild common HE, either chronic hemolysis or moderate hemolysis

• Common HE with severe infant pyropoikilocytosis shows moderate hemolysis

• Silent carrier

• Mild common HE, either chronic hemolysis or moderate hemolysis

• Common HE with severe infant pyropoikilocytosis shows moderate hemolysis somal dominant disorder has a well-defined band 3 molecular defect (Fig. 7.5).

Spherocytic Hereditary Elliptocytosis

This defect is a cross between HE and HS. The red cells of affected individuals will show more spherocytes and oval elliptocytes. This defect is common in individuals with northern European ancestry and shows a mild hemolysis and red cells of increased osmotic fragility. Gallbladder disease is a common feature, and splenec-tomy may be indicated if the hemoglobin drops quickly.

Hereditary Pyropoikilocytosis

This rare recessive disorder of the red cell membrane affects African American individuals primarily. Two mechanisms are at work in the red cells of HPP: a reduced assembly of alpha and beta spectrin on the membrane and increased susceptibility of mutant spec-trin to degrade.12 Hemoglobin values are extremely low, less than 6.5 g/dL, and the red cell morphology is incredibly bizarre with red cell budding, rare ellipto-cytes, and spherocytes. What makes these defective red cells unique is their heat sensitivity. Normal red cells will

Figure 7.4 Elliptocytes. Note these cells are pencil shaped.

Figure 7.5 Photomicrograph of Southeastern Asian ovalocytosis.

Figure 7.4 Elliptocytes. Note these cells are pencil shaped.

Figure 7.5 Photomicrograph of Southeastern Asian ovalocytosis.

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