溶血性贫血-课件

单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,ppt课件,*,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,溶血性贫血,Hemolytic Anemia,1,ppt课件,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,2,ppt课件,Mechanisms,and Causes,INTRACORPUSCULAR HEMOLYSIS,Membrane Abnormalities,Metabolic Abnormalities,Hemoglobinopathies,EXTRACORPUSCULAR HEMOLYSIS,Nonimmune,Immune,3,ppt课件,Membrane Defects,Microskeletal defects,Hereditary spherocytosis,Increased sensitivity to complement,Paroxysmal nocturnal hemoglobinuria,4,ppt课件,Enzymopathies,Glucose 6-Phosphate Dehydrogenase Deficiency,Pyruvate Kinase Deficiency,5,ppt课件,Hemoglobinopathies,Hemoglobinopathy,Thalassemia,6,ppt课件,Extracorpuscular HemolysisNonimmune,Infectious,Chemical,Thermal,Osmotic,7,ppt课件,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,8,ppt课件,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,9,ppt课件,Classification,Intravascular,E,xtravascular,10,ppt课件,clinical features,chronic,pallor,、,jaundice,、,splenomegaly,cholelithiasis,aplastic crisis,acute,algor,、,hyperpyrexia,、,sore waist,hemoglobinuria,jaundice,、,anaemia,11,ppt课件,laboratory examination,Increased RBC destruction,R,educed,RBC,life span,Indirect,h,yperbilirubinemia,E,rythroid hyperplasia,Reticulocytosis,E,rythroblasts,anisopoikilocytosis,spherocytes,in b,lood smear,E,rythroid hyperplasia,in bone marrow,12,ppt课件,13,ppt课件,diagnosis and differential diagnosis,hemolysis or not?,type of hemolysis,another anemia?,another jaundice with anemia?,another jaundice without anemia?,14,ppt课件,Treatment,Remove the causes,Immunosupressive,drug,W,ashed RBC transfusion,Splenectomy,Symptomatic treatment,15,ppt课件,Hereditary Spherocytosis,Defective or absent spectrin molecule,Leads to loss of RBC membrane,leading to spherocytosis,Decreased deformability of cell,Increased osmotic fragility,Extravascular hemolysis in spleen,16,ppt课件,Hereditary Spherocytosis,Pathophysiology,-,RBC,membrane protein defects(spectrin deficiency)resulting cytoskeleton instability,Familly history,Clinical features,splenomegaly,17,ppt课件,Hereditary Spherocytosis,Laboratory features,-hemolytic anemia -blood smear-microspherocytes -abnormal osmotic fragility test -positive autohemolysis test -prevention of increased autohemolysis by including,glucose in,incubation medium,Treatment,-,splenectomy,18,ppt课件,Hereditary Spherocytosis,19,ppt课件,Hereditary Spherocytosis,Osmotic Fragility,20,ppt课件,Paroxysmal Nocturnal Hemoglobinuria(PNH),PNH is an acquired chronic hemolytic anemia which arises from a somatic mutation in a hematopoietic stem cell.Most hematopoitic cell lines may be affected by the intrinsic membrane defect.This defect renders the red cells highly susceptible to complement mediated lysis resulting in the characteristic hemolysis.,21,ppt课件,Paroxysmal Nocturnal Hemoglobinuria(PNH),Pathogenesis,-an acquired clonal disease,arising from a somatic mutation in a single abnormal stem cell -glycosyl-phosphatidyl-inositol(GPI)anchor abnormality -deficiency of the GPI anchored membrane proteins(CD55 and CD59)-red cells are more sensitive to the lytic effect of complement -intravascular hemolysis,22,ppt课件,urine aliquot of PNH,23,ppt课件,clinical manifestation,pancytopenia,passage of dark brown urine in the morning,venous thrombosis(Budd-Chiari Syndrome),24,ppt课件,Laboratory features,Pancytopenia,C,hronic urinary iron loss,S,erum iron concentration decreased,Hemoglobinuria,Hemosiderinuria,P,ositive Ham,s test(acid hemolysis test),sugar-water test,S,pecific immunophenotype of,b,lood cells,(CD59,CD55),25,ppt课件,Attention,AA-PNH syndrome,AAPNH,PNHAA,PNH with AA,AA with PNH,26,ppt课件,Treatment,avoid causes,washed RBC transfusion,iron therapy,allogenic bone marrow transplantation,27,ppt课件,G-6-PD Deficiency,acute hemolytic anemia,congenital nonspherocytic hemolytic anemia,neonatal hyperbilirubinemia,（,kernicterus,）,favism,28,ppt课件,Level needed for protection,vs,ordinary oxidative stress,29,ppt课件,Autoimmune Hemolysis,Due to formation of autoantibodies that attack patient,s own RBC,s,Type characterized by ability of autoantibodies to fix complement&site of RBC destruction,Often associated with either lymphoproliferative disease or collagen vascular disease,30,ppt课件,Autoimmune Hemolytic Anemia,warm-reactive antibodies,primary,seco