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Hematology
Spherocytosis - Slide 1
Spherocytosis is an inherited hemolytic anemia caused by defects in erythrocyte membrane proteins, such as spectrin, ankyrin, or band 3. Due to a loss of membrane surface area, erythrocytes take on a spherical appearance (spherocytes) instead of the normal biconcave shape. These cells are less deformable and are broken down more rapidly in the spleen, leading to extravascular hemolysis.
Patients may present with anemia, jaundice, splenomegaly, and an increased risk of gallstones. Laboratory tests often reveal an elevated reticulocyte count, elevated bilirubin, and an elevated MCHC. Spherocytes are observed in the blood smear: small, round erythrocytes without a central clearing. The diagnosis can be confirmed with specialized tests, such as the EMA binding test or osmotic fragility test.
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Spherocytes
Spherocytosis
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