陈勤奋－溶血性贫血.ppt

溶血性贫血Hemolytic Anemia
华山医院血液科
陈勤奋
HEMOLYTIC ANEMIA (reduced RBC life span)
Anemia of increased destruction
Normochromic, normochromic anemia
Shortened RBC survival
Reticulocytosis--Response to increased RBC destruction
Increased indirect bilirubin
Increased LDH
Mechanisms and Causes
INTRACORPUSCULAR HEMOLYSIS
Membrane Abnormalities
Metabolic Abnormalities
Hemoglobinopathies
EXTRACORPUSCULAR HEMOLYSIS
Nonimmune
Immune
Membrane Defects
Microskeletal defects
Hereditary spherocytosis
Increased sensitivity plement
Paroxysmal nocturnal hemoglobinuria
Enzymopathies
Glucose 6-Phosphate Dehydrogenase Deficiency
Pyruvate Kinase Deficiency
Hemoglobinopathies
Hemoglobinopathy
Thalassemia
Extracorpuscular HemolysisNonimmune
Infectious
Chemical
Thermal
Osmotic
Extracorpuscular Hemolysis Immune
All require antigen-antibody reactions
Types of reactions dependent on:
Class of Antibody
Number & Spacing of antigenic sites on cell
Availability plement
Environmental Temperature
Functional status of reticuloendothelial system
Manifestations
Intravascular hemolysis
Extravascular hemolysis
Extracorpuscular Hemolysis Immune
bine with RBC, & either
plement cascade, &/or
Opsonize RBC for immune system
If 1, if all plement cascade is fixed to red cell, intravascular cell lysis occurs
If 2, &/or plement is only partially fixed, macrophages recognize Fc receptor of Ig &/or C3b plement & phagocytize RBC, causing extravascular RBC destruction
Classification
Intravascular
Extravascular