内科学 贫血总论-谢彦晖.ppt

ANEMIA HematologyDepartment HuashanHospital FudanUniversity ShanghaiXieYan Hui DIAGNOSISANDCLASSIFICATIONAnemiaisanabsolutedecreaseinhematocrit hemoglobinconcentration ortheRBCcount Anemiaisnotadiagnosis butasignofunderlyingdisease Hemoglobin Hb male adult 120g Lfemale adult 110g Lfemale gestation 100g LRedcellcountmale 4 5x1012 Lfemale 4 0 x1012 LHemocrit HCT male 0 42female 0 37female gestation 0 30 Determinationofthecause A History1 Drugadministration 2 Exposuretotoxicchemicals3 Familyoccurrence 4 Recenttransfusions5 menstruation woman 6 ingestion child 7 chronicdisease8 Ageatonset B Physicalfindingsandcomplaintsa Palemucousmembranesandskinsb Weakness lossofstamina andexerciseintolerance Hypersensitivitytocold fever c Tachycardiaandpolypnea Heartmurmur anemiaassociatedcardiacdisease Hb 30g Lmorethan2monthsheartenlargementSTdepression d headache dizzinesse anorexia nauxea abdominalfullnessdiarriaorconstipation Icterus f menstruationdisorderoramenorreahemoglobinuriag Shockif 1 2bloodvolumelostinshortperiod C Laboratoryfindings1 TheHctistheeasiest mostaccuratemethodfordetectinganemia Itsresultshouldbeinterpretedwithknowledgeofthehydrationstatusandanyalterationcausedbyspleniccontraction 2 HbandRBCmaybeusedtofurtherclassifytheanemia II ClassificationA Size MCV andHbConcentration MCHC 1 Normocytic macrocytic microcytic 2 Normochromic hypochromic Hyperchromiadoesnotoccur TypeMCV fl MCHC MCH pg disorderMacro 100 3232 35megaloblasticanemiaMDSNormo80 10026 3232 35aplasticanemia bloodlost hemolyticanemiaMicro 80 26 32irondeficiencyanemiasideroblasticanemiathalassemia B Bonemarrowresponse1 Regenerativea BonemarrowactivelyrespondsbyincreasingitsproductionofRBC s b Findings 1 Polychromasia 2 Reticulocytosis 3 Macrocytosis increasedMCV andhypochromiaassociatedwithreticulocytosis 4 HypercellularbonemarrowwithalowM Eratio 5 IncreaseinMCVandRDW c Thepresenceofregenerationsuggestsanextramarrowcause 1 Bloodloss 2 Erythrocytedestruction hemolysis d Bonemarrowexaminationwouldrevealerythropoietichyperplasia 2 Non Regenerativea Inadequatebonemarrowresponsebecauseofabonemarrowdisorder b Polychromasiaandreticulocytosisareabsent C PathophysiologicmechanismBloodloss hemorrhagicanemia 2 Acceleratederythrocytedestruction hemolyticanemia 3 Reducedordefectiveerythropoiesis ANEMIAFROMACCELERATEDERYTHROCYTEDESTRUCTION HEMOLYTICANEMIA A Clinicalfindings1 Clinicalsignsofhemorrhageareabsent 2 Jaundicemaybeseeninacuteandseverecases 3 Hemoglobinuriaandredplasmaisseenifsignificantintravascularhemolysisoccurs B Laboratoryfindings1 Reticulocytecountsarehigherinhemolyticanemiasthanexternalhemorrhagicanemias2 Plasmaproteinconcentrationisnormalorincreased 3 Neutrophilicleukocytosisandmonocytosismayoccur 4 EvidenceofHbdegradation hyperbilirubinemia hemoglobinuria 5 Abnormalerythrocytemorphology Heinzbodies erythrocyticparasites spherocytes orpoikilocytes I DifferentiationoftheCausesofHemolyticAnemiasA Extravascularhemolysis 1 MechanismsAutoimmuneMediated Antibodyand orC3mediated AIHA infection drug immunesystemdisorder b Decreasederythrocytedeformability a Shistocytesofmicroangiopathicanemia b Spherocytesofimmune mediatedanemia c Parasitizederythrocytes d Heinzbody containingcells c ReducedglycolysisandATPcontentoftheerythrocyte PKdeficiency d Increasedmacrophageactivity hypersplenism e Intravascularcausesofhemolysisdonotlyseallerythrocytes somealteredcellsmayremainthatareremovedbyphagocytosis 2 Clinicalandlaboratorycharacteristicsofphagocytic extravascular hemolysis Usuallychronicwithinsidiousonset b Aregenerativeresponse c Hemoglobinemiaandhemoglobinuriaareabsent d Hyperbilirubinemiae Neutrophilia monocytosis andthrombocytosisf Splenomegaly h Low gradeextravascularhemolysisoccursinmanyanemiasthatareprimarilynonhemolytic e g anemiaofchronicrenaldisease iron deficiencyanemia Referredtoasthe hemolyticcomponent ofothertypesofanemia B Intravascularhemolysis Erythrocytesaredestroyedwithinthecirculation releasinghemoglobinintotheplasmawhereitiseitherremovedbytheliverorexcretedbythekidneys 1 Mechanisms TheerythrocytemembranemustbesignificantlydisruptedtoallowescapeoftheHbmoleculeintotheplasma Mostofthemechanismsofintravascularhemolysisareextrinsicorextracorpusculardefects theerythrocyteisinitiallynormal Complement mediatedlysis neonatalisoerythrolysisandtransfusionreactions PNH b Physicalinjury Traumatic microangio pathicanemia DIC Coagulation Vasculitis c Oxidativeinjury Heinzbody methemo globin d Osmoticlysis hypotonicintravenousfluids e Othermembranealterations 1 Castorbeans ricin Causesdirectlysis 2 Snakevenoms 3 Bacterialtoxins 4 Parasites Babesia 2 Clinicalandlaboratorycharacteristicsofintravcascularhemolyticanemia Mostcasespresentasperacuteoracuteepisodes b Historymayrevealexposuretocausativedrugsorplants recenttransfusionofblood orrecentingestionofcolostrum c Aregenerativeresponseoccurs butitmaynotbeevidentinearlystages d Hemoglobinemiaistheprincipalfeatureofintravascularhemolysis 1 Reddiscolorationofplasma 2 IncreasedMCHCe Hemoglobinuriaf Hemosiderinuriag Hyperbilirubinemia h Additionallaboratoryfindingsmayincludeschistocytes keratocytes Heinzbodies erythrocyticparasites positiveCoombs test ANEMIAFROMREDUCEDORDEFECTIVEERYTHROPOIESISreducedordefectiveerythropoisislongoronsetinsidiouscliniccourse I Generalconsiderations A Mechanisms 1 PrecursorcellsNutrients ironandBvitamins Stimulation erythropoietin 2 Bonemarrowfailure intramarrowdiseaseandextramarrowcauses 3 Bonemarrowfailuremaybeselectivefortheerythroidseriesormayalsoaffecttheothercelllines B BonemarrowresponseWhenthenumberofprecursorcellsorerythropoieticstimulationisinadequate theerythroidmarrowishypocellular 2 Maturationabnormalitieswhichcharacterizethenutritionaldeficiencies areassociatedwithaHypercellularmarrowandineffectiveerythropoiesis 3 Alldegreesofbonemarrowfailurecanoccur fromcompleteaplasiatoasuboptimalresponseoftheerythroidmarrowfollowinghemorrhageorhemolysis II Differentiationofanemiascausedbyreducedordefectiveerythropoiesis erythrocytemorphology bloodneutrophilplateletnumbersbonemarrowcellularity A Normocytic normochromicanemia normalorincreasedneutrophilandplateletnumbers increasedM Eratiocausedbyhypocellularerythroidmarrow 1 Anemiaoferythropoietinlack a Chronicrenaldisease Anemiaproportionaltoseverityoftheuremia b Endocrinopathies 1 Cushings 2 Hypoandrogenism 3 Hypopituitarism 2 Anemiaofchronicdisorders ACD a Occursinchronicinfectious inflammatory orneoplasticdisorders b Cytokinesinvolvedwiththeinflammatoryprocessinitiatetheanemia c Erythrocytelifespanreduced d Laboratoryfindingsinclude 1 Lowserumiron 2 Lowtotalironbindingcapacity 3 Increasedbonemarrowmacrophageiron 4 Mild moderateanemiathatisusuallynonprogressive 3 Pureredcellaplasiaa Characterizedbyaselectivelossoferythroidprecursorsinthebonemarrow b Thoughttobeimmunemediated 4 Unknownmechanismsa Liverdiseaseb VitaminEdeficiency B Normocytic normochromicanemia neutropeniaand orthrombocytopenia M Eratioisdifficulttodeterminebecauseofhypocellularity 1 Aplasticanemiaa pancytopenia b shorterlifespansofthecells c Causes 1 Drugs chemicals plants 2 Irradiation 3 CytotoxicTcellsorantibody 4 Infectiousagents 2 Myelophthisicanemiaa Thebonemarrowisphysicallyreplacedbyanabnormalproliferationofcells 1 Myeloproliferativedisorders leukemias 2 Myelofibrosis 3 Osteosclerosis 4 Diffusegranulomatousosteomyelitis 5 Metastaticcancer 3 Anemiacausedbyinfectiousagentsa Ehrlichiosis 埃里西提病 b FeLV 猫白血病病毒 C Microcytic hypochromicanemia variableneutrophilandplateletnumber usuallyahypercellularmarrowwithavariableM Eratio 1 Irondeficiencya Chronichemorrhageb Dietarydeficiency especiallyinyoungmilk fedc Ineffectiveerythropoiesisearly d Laboratoryfindings 1 Lowserumiron 2 Variableiron bindingcapacity 3 Microcytosis 4 Hy