复旦大学临床医学专业：《内科学》课程教学资源（PPT课件讲稿）溶血性贫血（陈勤奋）

溶血性贫血 Hemolytic Anemia 山医血科 陈勤春

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 ORNormochrom1c. normochromic anemla SHOrtened rBc survival CRReticulocytosis--Response to increased rbc destruction INCreased indirect bilirubin INcreased LDh

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 aR⊥ mmune

Mechanisms and Causes ❖INTRACORPUSCULAR HEMOLYSIS Membrane Abnormalities Metabolic Abnormalities Hemoglobinopathies ❖EXTRACORPUSCULAR HEMOLYSIS Nonimmune Immune

Membrane Defects Microskeletal defects cOHereditary spherocytosis g Increased sensitivity to complement paRoxysmal nocturnal hemoglobinuria

Membrane Defects ❖Microskeletal defects Hereditary spherocytosis ❖Increased sensitivity to complement Paroxysmal nocturnal hemoglobinuria

Enzymopathies .Glucose 6-Phosphate Dehydrogenase Deficiency o Pyruvate Kinase Deficiency

Enzymopathies ❖Glucose 6-Phosphate Dehydrogenase Deficiency ❖Pyruvate Kinase Deficiency

Hemoglobinopathies Hemoglobinopathy Thalassemia

Hemoglobinopathies ❖Hemoglobinopathy ❖Thalassemia

Extracorpuscular Hemolysis nonimmune g Infectious ☆ Chemical ◆ Thermal Osmotic

Extracorpuscular Hemolysis Nonimmune ❖Infectious ❖Chemical ❖Thermal ❖Osmotic

Extracorpuscular Hemolysis Immune .All require antigen-antibody reactions .o Types of reactions dependent on: oR Class of Antibody oR Number spacing of antigenic sites on cell aVAilability of complement cR Environmental Temperature cR Functional status of reticuloendothelial system ☆ Manifestations iNTravascular hemolysis eXTravascular hemolysis

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 1. Activate complement cascade, &/or 2. Opsonize rbc for immune system %o If 1, if all of complement cascade is fixed to red cell. intravascular cell ysIs occurs &o 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

Extracorpuscular Hemolysis Immune ❖ Antibodies combine with RBC, & either 1. Activate complement cascade, &/or 2. 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

Classification ❖Intravascular ❖Extravascular