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,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,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,溶血性贫血Hemolytic Anemia1ppt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,HEMOLYTIC ANEMIA (reduced RBC,Mechanisms,and Causes,INTRACORPUSCULAR HEMOLYSIS,Membrane Abnormalities,Metabolic Abnormalities,Hemoglobinopathies,EXTRACORPUSCULAR HEMOLYSIS,Nonimmune,Immune,3,ppt课件,Mechanisms and CausesINTRACORP,Membrane Defects,Microskeletal defects,Hereditary spherocytosis,Increased sensitivity to complement,Paroxysmal nocturnal hemoglobinuria,4,ppt课件,Membrane DefectsMicroskeletal,Enzymopathies,Glucose 6-Phosphate Dehydrogenase Deficiency,Pyruvate Kinase Deficiency,5,ppt课件,EnzymopathiesGlucose 6-Phospha,Hemoglobinopathies,Hemoglobinopathy,Thalassemia,6,ppt课件,HemoglobinopathiesHemoglobinop,Extracorpuscular HemolysisNonimmune,Infectious,Chemical,Thermal,Osmotic,7,ppt课件,Extracorpuscular HemolysisNon,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 Im,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,Extracorpuscular Hemolysis Im,Classification,Intravascular,E,xtravascular,10,ppt课件,ClassificationIntravascular10p,clinical features,chronic,pallor,、,jaundice,、,splenomegaly,cholelithiasis,aplastic crisis,acute,algor,、,hyperpyrexia,、,sore waist,hemoglobinuria,jaundice,、,anaemia,11,ppt课件,clinical featureschronicacute1,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,laboratory examinationIncrease,13,ppt课件,13ppt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,diagnosis and differential dia,Treatment,Remove the causes,Immunosupressive,drug,W,ashed RBC transfusion,Splenectomy,Symptomatic treatment,15,ppt课件,TreatmentRemove the causes 15p,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 SpherocytosisDefect,Hereditary Spherocytosis,Pathophysiology,-,RBC,membrane protein defects (spectrin deficiency) resulting cytoskeleton instability,Familly history,Clinical features,splenomegaly,17,ppt课件,Hereditary SpherocytosisPathop,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 SpherocytosisLabora,Hereditary Spherocytosis,19,ppt课件,Hereditary Spherocytosis19ppt课,Hereditary Spherocytosis,Osmotic Fragility,20,ppt课件,Hereditary Spherocytosis Osmo,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 Hemoglobi,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,Paroxysmal Nocturnal Hemoglobi,urine aliquot of PNH,23,ppt课件,urine aliquot of PNH23ppt课件,clinical manifestation,pancytopenia,passage of dark brown urine in the morning,venous thrombosis(Budd-Chiari Syndrome),24,ppt课件,clinical manifestationpancytop,Laboratory features,Pancytopenia,C,hronic urinary iron loss,S,erum iron concentration decreased,Hemoglobinuria,Hemosiderinuria,P,ositive Hams test (acid hemolysis test),sugar-water test,S,pecific immunophenotype of,b,lood cells,(CD59, CD55),25,ppt课件,Laboratory featuresPancytopeni,Attention,AA-PNH syndrome,AAPNH,PNHAA,PNH with AA,AA with PNH,26,ppt课件,AttentionAA-PNH syndrome26ppt课,Treatment,avoid causes,washed RBC transfusion,iron therapy,allogenic bone marrow transplantation,27,ppt课件,Treatmentavoid causes27ppt课件,G-6-PD Deficiency,acute hemolytic anemia,congenital nonspherocytic hemolytic anemia,neonatal hyperbilirubinemia,（,kernicterus,）,favism,28,ppt课件,G-6-PD Deficiencyacute hemolyt,Level needed for protection,vs,ordinary oxidative stress,29,ppt课件,Level needed for protection vs,Autoimmune Hemolysis,Due to formation of autoantibodies that attack patients own RBCs,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 HemolysisDue to for,Autoimmune Hemolytic Anemia,warm-reactive antibodies,primary,secondary,cold,-reactive antibodies,cold agglutinin syndrome,paroxysmal cold hemoglubinuria,31,ppt课件,Autoimmune Hemolytic Anemiawar,Autoimmune hematolysisWarm Type,IgG+C3,IgG,C3,32,ppt课件,Autoimmune hematolysisWarm Ty,Autoimmune hematolysis Warm Type,Usually IgG antibodies,Fix complement only to level of C3,if at all,Immunoglobulin binding occurs at all temps,Fc receptors/C3b recognized by macrophages,Hemolysis primarily extravascular,70% associated with other illnesses,Responsive to steroids/splenectomy,33,ppt课件,Autoimmune hematolysis Warm T,Clinical manifestation,anemia,、,jaundice,、,splenohepatomegalia,ITP+AIHA=Evens syndrome,34,ppt课件,Clinical manifestationanemia、j,Laboratory examination,Blood：anemia；Ret,；,e,rythroblasts,anisopoikilocytosis,Bone marrow：e,rythroid hyperplasia,Coombs Test,35,ppt课件,Laboratory examinationBlood：an,Coombs Test - Direct,Looks for immunoglobulin &/or complement of surface of red blood cell (normally neither found on RBC surface),Coombs reagent - combination of anti-human immunoglobulin & anti-human complement,Mixed with patients red cells; if immunoglobulin or complement are on surface, Coombs reagent will link cells together and cause agglutination of RBCs,36,ppt课件,Coombs Test - DirectLooks for,Coombs Test - Indirect,Looks for anti-red blood cell antibodies in the patients serum, using a panel of red cells with known surface antigens,Combine patients serum with cells from a panel of RBCs with known antigens,Add Coombs reagent to this mixture,If anti-RBC antigens are in serum, agglutination occurs,37,ppt课件,Coombs Test - IndirectLooks fo,Diagnosis,38,ppt课件,Diagnosis38ppt课件,Treatment,Steroids,Splenectomy,I,mmunosupressive agents,T,ransfusion,39,ppt课件,TreatmentSteroids39ppt课件,Autoimmune hematolysis Cold Type,Most commonly IgM mediated,Antibodies bind best at 30 or lower,Fix entire complement cascade,Leads to formation of membrane attack complex, which leads to RBC lysis in vasculature,Typically only complement found on cells,90% associated with other illnesses,Poorly responsive to steroids, splenectomy; responsive to plasmapheresis,40,ppt课件,Autoimmune hematolysis Cold T,Hemolytic anemia,Summary,Myriad causes of increased RBC destruction,Marrow function usually normal,Often requires extra folic acid to maintain hematopoiesis,Anything that turns off the bone marrow can result in acute, life-threatening anemia,41,ppt课件,Hemolytic anemiaSummaryMyriad,