陈勤奋－溶血性贫血.pptVIP

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 to complement 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 of complement Environmental Temperature Functional status of reticuloendothelial system Manifestations Intravascular hemolysis Extravascular hemolysis Extracorpuscular Hemolysis Immune Antibodies combine with RBC, either Activate complement cascade, /or Opsonize RBC for immune system If 1, if all of complement cascade is fixed to red cell, intravascular cell lysis occurs If 2, /or if complement is only partially fixed, macrophages recognize Fc receptor of Ig /or C3b of complement phagocytize RBC, causing extravascular RBC destruction Classification Intravascular Extravascular clinical features chronic pallor 、 jaundice 、 splenomegaly cholelithiasis aplastic crisis acute algor、hyperpyrexia、sore waist hemoglobinuria jaundice、anaemia laboratory examination Increased RBC destruction Reduced RBC life span Indirect hyperbilirubinemia Erythroid hyperplasia Reticulocytosis Erythroblasts, anisopoikilocytosis, spherocytes in blood smear Erythroid hyperplasia in bone marrow diagnosis and differential diagnosis hemolysis or not? type of hemo