溶血性贫血讲义.ppt

Hereditary HA Membrane abnormalities Enzymopathies Plasma constituent abnormalities RBC Membrane Structure + + Plasma IntegralProteins Peripheral Proteins Lipid Bi-Layer — — Lipid Bi-Layer RBC Cytoplasm Membrane Abnormalities Spherocytosis Elliptocytosis Pyropoikilocytosis Stomatocytosis Membrane Abnormalities See figure 17-1 Excess membrane cholesterol RBC inclusions Senescent RBC Complement coated RBC Antibody coated RBC Rigid RBC Spleen: Effects on RBCs Hereditary Spherocytosis Defect: RBC protein defects affecting spectrin Pathophysiology Increased Na+ permeability ? RBC rigidity ? Destruction in spleen Testing: Increased osmotic fragility Incubated osmotic fragility Hereditary Spherocytosis See figures 17-2 and 3 Spherocyte Morphology Pathophysiology Lacks area of central pallor Additional causes Immunologic reaction Physical trauma Usually 6.5 ?m diameter Microspherocytes: 4 ?m diameter Hereditary Elliptocytosis Defect: Unknown; spectrin defects? Pathophysiology Increased Na+ efflux ? Hemoglobin bipolarization; Cholesterol polarization ? RBC rigidity ? Destruction in spleen Ellipto/Ovalocyte Clinical Relevance Hereditary ellipto- or ovalocytosis (25% of all RBCs) Macro-ovalocytes seen in megaloblastic anemias Non-specific finding in other types of anemia(25% of all RBCs) Hereditary Elliptocytosis Testing: Morphology Increased osmotic fragility Hereditary Elliptocytosis Slides not shown. See Figure 8-7, p. 91, Clinical Hematology Hereditary Pyropoikilocytosis Defect: Spectrin defect Pathophysiology: RBC rigidity Destruction in spleen Testing: Increased thermal sensitivity test Hereditary Pyropoikilocytosis Slides not shown. See Figure 8-15, p. 95, Clinical Hematology Hereditary Stomatocytosis Defect: Unknown Pathophysiology: Increased influx of RBC H2O RBC rigidity Destruction in spleen Stomatocyte Morphology Pathophysiology Slit-like area of central pallor Hereditary Stomatocytosis Testing: Morphology Stomatocytosis: Increased osmotic fragility Xerocytosi