内科学 溶血性贫血(英文)

HemolyticAnemia

1CONTENTSDEFINITIONCLINICALCLASSIFICATIONCLINICALMANIFESTATIONLABORATORYEXAMINATIONDIAGNOSIS&THERAPY2DEFINITIONHemolyticanemiaresultsfromshortenedRBCsurvivalAnincreasedrateandspeedinRBCdestruction.BMhasthecapacitytocompensateproductionofRBCfrom6-8folds.HemolyticdiseasehasapersistentlyelevatedreticulocytecountwithanormalHb.3CONTENTSDEFINITIONCLINICALCLASSIFICATIONCLINICALMANIFESTATIONLABORATORYEXAMINATIONDIAGNOSIS&THERAPY4CLINICALCLASSIFICATIONHemolyticsite

1.Intravascularhemolysis:

RBCareactuallylysedinthecirculationandbloodvessels.PNH2.Extravascularhemolysis:

RBCarephagocytosedbyreticuloendothelialsystemandremovedfromthecirculationbecauseofinflexibleprematurely.AIHA,HS.

5Insituhemolysis:

RBCaredestructedinBMbeforetheyarereleasedintoblood6MembraneEnzymeHemoglobinExtrinsicfactorsMaintainingRBCcytotostaticfactors7Mechanisms

1.RBCintrinsicabnormal

Membraneabnormalities

Hereditary

spherocytosisParoxysmalnocturnalhemoglobinuriaEnzymeabnormalities

Glucose6-phosphatedehydrogenasedeficiencyPyruvatekinasedeficiencyHemoglobinabnormalities

HemoglobinopathyThalassemia

82.RBCextrinsicabnormal(usuallyacquired)

Immuneinducedautoimmunity:AIHAalloimmunity:bloodtypeincompatibility,hemolyticdiseaseofthenewbornVascularfactorsinduced

marchhemoglobinuria,DIC,heartvalvesurgeryBiologicalfactorsmediated

malaria,Assamfever,snakevenomsphysicalandchemicalfactorsmediated

burns,toxication9CONTENTSDEINITIONCLINICALCLASSIFICATIONCLINICALMANIFESTATIONLABORATORYEXAMINATIONDIAGNOSIS&THERAPY10CLINICALFEATURESChronicpallor

、

jaundice

、splenomegalycholelithiasisliverfunctionlesionboneaplasticAcuteMostcasespresentasperacuteoracuteepisodesAlgor、highfeverwaistpainhemoglobinuria、Jaundicenauseaandvomitcoma、shockcongestiveheartfailurerenalfailure111213141516CONTENTSDEFINITIONCLINICALCLASSIFICATIONCLINICALMANIFESTATIONLABORATORYEXAMINATIONDIAGNOSIS&THERAPY17RBCreticuloendothelialsystemHbglobinironporphyrin(unconjugatedbilirubin)

liverglucuronicacid

conjugatedbilirubin

bile

intestinaltract

stercobilinogen

stool

(stercobilinogen)enterobacteriakidney

urine

(urobilinogen)

(enterohepaticcirculation)（Spleen）Extravascularhemolysis18

liberationHb↑

ferrihemoglobin(hemoglobinemia)

hematinalbumin

(hematinalbuminaemia)oxydation

liver

bileHaptoglobin-hemoglobincombined

haptoglobinkidneyglomerulirenisrenaltubulehematoglobinuriaurinarysiderosisIntravascularhemolysis19IncreasedRBCdestructionErythroidhyperplasiaReducedRBClifespanLaboratoryexamination20BloodroutintestNormochromicanemiaNeutrophilia,monocytosisthrombocytosis21IntravascularhemolysisExtravascularhemolysisOtherevidences1.hyperbilirubinemia2.increasedstercobilinogen3.Increasedurobilinogen1.hemoglobinemia2.decreasedhaptoglobin3.hematoglobinuria4.urinarysiderosis5.hyperbilirubinemia6.increasedstercobilinogen7.Increasedurobilinogen1.increasedLDH2.

fragmentIncreasedRBCdestruction2223ErythroidhyperplasiaReticulocytosis(atleast>5%)ErythroblastsErythroidhyperplasiainbonemarrow2425262728ReducedRBClifespanAbnormalerythrocytemorphology:poikilocytes,anisopoikilocytosis,spherocytesinbloodsmear2.PhagocytizedRBC,autoagglutination3.Heinzbody4.Increasedosmoticfragility5.ShortenedRBCsurvivaltime293031323334CONTENTSDEFINITIONCLINICALCLASSIFICATIONCLINICALMANIFESTATIONLABORATORYEXAMINATIONDIAGNOSIS&THERAPY35DIAGNOSISHemolyticAnemiaclinicalsituationtypeorcauseofhemolysishemolysisornot

36

(+)—autoimmunehemolyticanemia,AIHA

RBCextrinsicfactors:chemistry、physics、

(-)

infection、immunity

RBCintrinisicfactors:

osmoticfragilitytest

increased

decreased

normal

membrane

HemoglobinEnzyme

CoombsTest37TreatmentRemovethecausesImmunosupressivedrug:CsA,danazol,azathioprinCorticosteroid:prednisone,DXM,methylprednisoloneDosage:30-60mg/dtoHbelevatedtonormal,graduallydropdownonceaweekandthenmaintainwithlowdose(5-10mg/d)for3-6monthsSide-effect:hyperglycemia,gestro-uncler,hypertention,sodium-waterretention38Bloodtransfusion:washedRBC,RBC,transfusionshouldbematchedwellinABOandRhtype.SplenectomySymptomatictreatment39G-6-PDDeficiencyacutehemolyticanemiacongenitalnonspherocytichemolyticanemianeonatalhyperbilirubinemia（kernicterus）favism40AutoimmuneHemolyticAnemiaDuetoformationofautoantibodiesthatattackpatient’sownRBC’sTypecharacterizedbyabilityofautoantibodiestofixcomplement&siteofRBCdestructionOftenassociatedwitheitherlymphoproliferativediseaseorcollagenvasculardisease41AutoimmuneHemolyticAnemiawarm-reactiveantibodiesprimarysecondarycold-reactiveantibodiescoldagglutininsyndromeparoxysmalcoldhemoglubinuria42Autoimmunehematolysis

(warmtype)UsuallyIgGantibodiesFixcomplementonlytolevelofC3,ifatallImmunoglobulinbindingoccursatalltempsFcreceptors/C3brecognizedbymacrophagesHemolysisprimarilyextravascular70%associatedwithotherillnessesResponsivetosteroids/splenectomy43Autoimmunehematolysis

(coldtype)