1、教学贫血教学贫血第1页/共43页Definition A reduction below normal in the concentration of hemoglobin,the mass of red blood cells and/or the hematocrit in the blood.In men Hb 120g/L RBC 4.51012/L HCT 0.42 In women Hb 110g/L RBC4.0 1012/L HCT0.37 第2页/共43页Factors influencing Hb concentrationSexAgeAltitudeAlterations
2、 in plasma volume第3页/共43页Classification Morphologic classificationTypeMCV(um)MCH(PG)MCHC(%)DiagnosisMacrocytic1003232-35Megaloblastic anemiaNormocytic80-10026-3232-35AA,HA,Acute posthemorragic anemiaMicrocytic802632IDA,Sideroblastic anemiaThalassemias第4页/共43页Kinetic classification(Classified accordi
3、ng to etiology and pathogenesis)一、一、Decreased erythropoiesisDecreased erythropoiesis 1Proliferation and differentiation abnormalities of SCHSC:AA,Fanconis anemia,MDSErythrocytic progenitors:Pure red cell aplasia,Anemias caused by kidney failure and endocrine disorders第5页/共43页2Marrow infiltration leu
4、kemiascarcinomamultiple myeloma myelofibrosisMalignant histocytosis第6页/共43页3Production and maturation blockages of differentiating cells DNA synthesis blockage:Vit B12,falic acid difficiencies,嘌呤和嘧啶嘌呤和嘧啶 metabolic defectsMegaloblastic anemiaHb synthesis blockage:Heme production defect:IDA Heptoglobi
5、n production defect:Thalassemias第7页/共43页Clinical manifestationsGeneral manifestations:(Nonspecific and reflect tissue hypoxia)Tiredness,fatigue the most common,the earliest symptom Pallor of skin and mucosashared chracteristicCardiovascular systems:Palpitations and dyspnea on exertion,breathlessness
6、common symptomsValvulae murmurs Hb 3mAnemic cardic diseaseHereditary spherocytosisAIHAHypersplenism由于胸腺瘤(Thymoma)引起的纯红AA，切除胸腺可使部分患者病情缓解。The aged patients or patients with vascular or cardiac diseases may not stand to milder anemia.1）Endogenous(intra-erythrocyte defects)1.Androgens:may promate reythr
7、opoiesis:三、Laboratory findingsMechanical:march hemoglobinuria,cardiac valves prostheses,microangiopathic hemolytic disorders.ThalassemiasDue to chemical,physical or microrganisms:Chemical toxin-and drug-induced hemolysis,large scale burned patient(severe burned patient)Infection-induced,benzene,radi
8、ationChronic ITP5)Immunologic testsProliferation and differentiationClassificationMegaloblastic anemia,IDA Hypoplastic anemias(增生减低性贫血):AAProliferation and differentiation2)Tests for hemolytic anemia such as Coombstest4 Unknown reason or several mechanismsSideroblastic anemiaChronic disease anemias(
9、chronic inflummation,infections,尿毒症尿毒症,Hepatic disorders,neoplasm,connective tissue disease,endocrine disorders)第8页/共43页二、二、Accelerated destruction of red cells 1）Endogenous(intra-erythrocyte defects)1.Membrane defects of erythrocytes:Hereditary:Hereditary spherocytosis(HS)Hereditasy elliplocytosis(
10、HE)Acquired:PNH2.Enzyme defects Glucose-6-Phosphate Dehydrogenase (G6PD)deficiency Pyruvate kinase deficiency 3.Abnormal haptogllbin synthesis:Sickle cell anemia,Hemoglobinopathies Thalassemias第9页/共43页2)Extragenous1.Immune HA:AIHA,neonatal HA,mismatched transfusion,drug-induced HA2.Mechanical:march
11、hemoglobinuria,cardiac valves prostheses,microangiopathic hemolytic disorders.3.Due to chemical,physical or microrganisms:Chemical toxin-and drug-induced hemolysis,large scale burned patient(severe burned patient)Infection-induced,benzene,radiationmicrorganisms:Malaria,virus,etc.4.Increased damage o
12、f monocyte-macrocyte system:Hypersplenism第10页/共43页三三.Blood lossAcute posthemorrage anemiaChronic bleeding IDA第11页/共43页Classified accordind to the proliferative situations of BMHyperplastic anemias(增生性贫血):Hemolytic anemiaAnemia caused by blood lossMegaloblastic anemia,IDA Hypoplastic anemias(增生减低性贫血)
13、:AA第12页/共43页Clinical manifestationsFactors influencing symptoms:Severity of anemia Hb8090g/LsymptomsRapidity of anemia Abrupt loss of 20%of the circulating blood volume marked pallor,postural hypotension and tachycardia(心动过速心动过速)The loss of 50%severe shock even dealth.第13页/共43页Normal to high3 Neurol
14、ogic system:Headache,Dizziness,Confusion,decreased mental acuity(记忆力衰退),晕厥，注意力不集中，失眠，耳鸣 severe anemiaComa 4 Digestive system:Anorexia(食欲不振)，厌食early megaloblastic anemia Nausea(恶心)，flatulence(胀气)，diarrhoea(腹泻)or constipation(便秘)lingual abnormalities(舌的改变)：common1）Endogenous(intra-erythrocyte defects)
15、1.6)影像学检查：钡餐透视，钡灌肠一、Decreased erythropoiesisIDA,Sideroblastic anemiaAA,HA,Acute posthemorragic anemia二、Accelerated destruction of red cellsHyperplastic anemias(增生性贫血):Hemolytic anemiaNormal to highIn contrast,the gradual loss(even Hb 3040g/L)of the circulating red cell mass in a patient with pernici
16、ous anemia may occur without any symptoms at all.第14页/共43页Why?Red cell 2,3 DPG(RBC内内2,3-二磷酸甘油酸）二磷酸甘油酸）In anemia the oxyhemoglobin dissociation curve usually shifts(右移右移)in a manner to increase the quantity of oxygen released in tissues without oppeciably altering the quantity of osygen bound in the
17、lungs.Red cell 2,3 diphophoglycerate(2,3 DPG)regularly increases in anemic patients to mediate this effect.Maximum elevation of RBC 2,3 DPG increases oxygen delivery only about 30 per cent,but this is a highly efficient form of compensation requiring no significant expenditure of energy.第15页/共43页 Th
18、e aged patients or patients with vascular or cardiac diseases may not stand to milder anemia.第16页/共43页The level of anemia at which symptoms occur is highly variable among individuals as would be expected from the widely differing degrees of physical activity,physical conditioning,circulatory adequac
19、y,and sensitivity or stoicism of the population.第17页/共43页General manifestations:(Nonspecific and reflect tissue hypoxia)Tiredness,fatigue the most common,the earliest symptom Pallor of skin and mucosashared chracteristicCardiovascular systems:Palpitations and dyspnea on exertion,breathlessnesscommon
20、 symptomsValvulae murmurs Hb 3mAnemic cardic disease第18页/共43页 3 Neurologic system:Headache,Dizziness,Confusion,decreased mental acuity(记忆力衰退记忆力衰退),晕厥，注意力晕厥，注意力不集中，失眠，耳鸣不集中，失眠，耳鸣 severe anemiaComa 4 Digestive system:Anorexia(食欲不振食欲不振)，厌食，厌食early megaloblastic anemia Nausea(恶心恶心)，flatulence(胀气胀气)，diar
21、rhoea(腹泻腹泻)or constipation(便秘便秘)lingual abnormalities(舌的改变舌的改变)：common第19页/共43页5Genitourinary system:severe anemiapolyurea(多尿多尿),hypobaric urine(尿比重低尿比重低),proteinuria(蛋白尿蛋白尿):肾小球滤过功能和小肾小球滤过功能和小管分泌及回收功能障碍管分泌及回收功能障碍female:disturbed menstruation(月经紊月经紊乱乱),性功能减退多见性功能减退多见6Other:7.Manifestations of underl
22、ying disease:第20页/共43页The process of correct diagnosis is the one of differential diagnosis.Steps:1.To Establish the type of anemia 2.To find out the cause or underlying diseases of anemiaDiagnosis第21页/共43页1.1.Cause or inducer of anemia:Cause or inducer of anemia:Nutrition,special habits for food ch
23、ange in stool habits:stool Guaiacs in all professioninfluation of surrounding environment chronic diseases menstruation,marriage一一History 第22页/共43页2.Developing processes,severity 2.Developing processes,severity&complications of anemias&complications of anemias Duration and onset of symptoms 3.Import
24、ant laboratory results,3.Important laboratory results,diagnosis,treatments and their diagnosis,treatments and their effects.effects.第23页/共43页skin and mucosa:pallor,jaundice,petechiaehair and nails adenopathy(淋巴结肿大淋巴结肿大)hepatomegaly-splenomegalyneurologic abnormalities 肛门及妇科盆腔检查肛门及妇科盆腔检查二二Physical ex
25、amination第24页/共43页、The hemogram The hemogram（血像）（血像）Routine blood exam(Hb,RBC)The reticulocyte count(RC)RC:hyperplasticRC:hypoplastic三、三、Laboratory findings第25页/共43页Reticulocyte Count Is required in the evaluation of all patients with anemia as it is a simple measure of production Young RBC that sti
26、ll contains a small amount of RNA Normally take 1 day for reticulocyte to mature.Under influence of epo takes 2-3 days 1/120th of RBC normally第26页/共43页Absolute Retic count Retic counts are reported as a percentage:RBC count x Retic%=Absoulte retic count normal:(77+23)x 109/L Absolute Retic counts ne
27、ed to be corrected for early release(If polychromasia is present)Absolute retic/2(for hct in mid 20s)Absolute retic/3(hct 20)第27页/共43页MCV(the mean corpuscular volume):the most useful of the RBC indices.MCH,MCHC:are rarely as helpful as the MCVThe leukocyte and platelet count:Anemia with a diminished
28、 leukocyteand platelet count-pancytopenia-suggests either primary marrow disease,megaloblastic anemia,or hypersplenism.Examination of the peripheral blood smear:第28页/共43页2.Examination of bone marrow2.Examination of bone marrow 骨髓涂片检查：主要观察骨髓涂片检查：主要观察BM增生程度，各系统细胞分类增生程度，各系统细胞分类计数，异常细胞，正常计数，异常细胞，正常BM组织有
29、核细胞与脂肪组织各占组织有核细胞与脂肪组织各占1/2，前者增多（尤其是红系）见于增生性贫血，后者增，前者增多（尤其是红系）见于增生性贫血，后者增多代表多代表BM增生低下，见于增生低下，见于AA，骨髓小粒是血液稀释与，骨髓小粒是血液稀释与否的一个重要标志。否的一个重要标志。Examination of aspirated smears in general gives superior cytologic information while the core biopsy provides crucial information concerning the overall cellularit
30、y,as well as the presence of fibrosis,tumor,or granulomas.Both procedures are complemantary and are best performed together when the diagnosis is in doubt.Bone marrow iron staining第29页/共43页3.Some useful ancillary tests3.Some useful ancillary tests1)Stools for occult blood 2)Tests for hemolytic anemi
31、a such as Coombstest3)Tests for nutritional anemia such as serum folic acid,serum Vitamin B12,SI,SF,etc.4)Liver and kidney function tests5)Immunologic tests6)影像学检查：钡餐透视，钡灌肠影像学检查：钡餐透视，钡灌肠第30页/共43页Treatment一、一、Treatment of the causesThe purpose is the treatment of the underlying disease.Gastric cancer
32、Pernicious anemia（恶性贫血）（恶性贫血）（megaloblastic anemia）第31页/共43页二、二、Drugs明确病因之前且忌乱投药明确病因之前且忌乱投药 Iron agents;calculation of dose falic acid,Vit B12 Vit B6 Corticosteroids:AIHA，AA,PNH Androgens:may promate reythropoiesis:1.)刺激刺激EPO分泌。分泌。2）增强）增强BM对对EPO的效应。的效应。EPO:Anemia caused by kidney diseases Immunosupp
33、ression agentsALG(antilymphocyte globulin）CsA:Acute and severe AA 第32页/共43页三、三、Transfusions Avoid of complications as hepatitis and AIDS Whole blood Frozed red cells Washed red cells Leukocyte poor packed cells（浓缩（浓缩RBC）Chronic anemia:Hb300)lowAnemia of Chronic DxlowlowNormal to highAplastic anemia
34、HighExtremely highNormal to high第36页/共43页Laboratory findings in anaemiaDrs Shepherd,Dexter,and Rapson Spring 2001第37页/共43页Scanning Electron microscopy:normal red cell第38页/共43页Classification Morphologic classificationTypeMCV(um)MCH(PG)MCHC(%)DiagnosisMacrocytic1003232-35Megaloblastic anemiaNormocytic
35、80-10026-3232-35AA,HA,Acute posthemorragic anemiaMicrocytic802632IDA,Sideroblastic anemiaThalassemias第39页/共43页2Marrow infiltration leukemiascarcinomamultiple myeloma myelofibrosisMalignant histocytosis第40页/共43页三三.Blood lossAcute posthemorrage anemiaChronic bleeding IDA第41页/共43页Why?Red cell 2,3 DPG(R
36、BC内内2,3-二磷酸甘油酸）二磷酸甘油酸）In anemia the oxyhemoglobin dissociation curve usually shifts(右移右移)in a manner to increase the quantity of oxygen released in tissues without oppeciably altering the quantity of osygen bound in the lungs.Red cell 2,3 diphophoglycerate(2,3 DPG)regularly increases in anemic patients to mediate this effect.Maximum elevation of RBC 2,3 DPG increases oxygen delivery only about 30 per cent,but this is a highly efficient form of compensation requiring no significant expenditure of energy.第42页/共43页五、五、BMT（bone marrow transplantation）SAA MDS第43页/共43页
