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AplasticanemiaCONTENTSINTRODUCTIONETIOLOGY/PATHOGENESISMANIFESTATIONSLABORATORYFINDINGSDIAGNOSIS&DIFFERENTIALTREATMENTAplasticanemia(AA)DefinitionAA,anunusualhematologicdisease,istheparadigmofthehumanbonemarrowfailuresyndromes.
-marrowhypoplasia
-peripheralpancytopeniaIncidence:7.4permillionpopulation
East>West
Biphasicpeakat20-25and>60yCauses:Inherited(20%)FanconianemiaDyskeratosiscongenitaCartilagehairhypoplasiaPearsonsyndromeAmegakaryocyticthrombocytopeniaShwachman-DiamondsyndromeDubowitzsyndromeDiamond-BlackfansyndromeFamilialaplasticanemiaCauses:Acquired(80%)
Infections:hepatitis,EBV,HIV,parvovirusRadiationandchemicalssuchasbenzeneDrugs:chloramphenicol,phenylbutazone,goldPNH
(paroxysmalnocturnalhemoglobinuria)TransfusionalgraftversushostdiseaseLivertransplantationPregnancyPathogenesisDepletionofhemopoieticstemcellSuppressionofproliferationandmaturation
Mechanism
-Immunerelated
-CytotoxicT-cell,INF(Interferon)γ,TNF(tumornecrosisfactor
)α↑PresentationAnemia:pallor,headache,palpitations,
dyspnea,heartfailureThrombocytopenia:mucosalandgingival
bleedingorpetechiaNeutropenia:infections,mouthand
pharyngealulcerationsTestsPB,BM(multiple)smear,biopsy,cytochemistryChromosomeTcellnumberandfunctionOthers
VirusAb(hepatitis,herpes,rubella…)
Coombstest(direct&indirect)
VitB12
ANA(antinuclearantibody),C3,C4
Hbelectrophoresis,
sugarwatertest
HLAtyping,BMchromosomeBMaspirationandbiopsyDIAGNOSISOFAACriteria
directevidencesexclusiveevidencesSubtypes
SAA(severeAA)
VSAA(verysevereAA)NSAA(notsevereAA)CRITERIAOFAADIRECTEVIDENCES:PancytopeniaHypocellularityofBM3myeloidlineagesobviouslyreducedinBMIncreasedpercentagesandfunctionofTlymphocytesNormalmorphologicshapesofPBandBMcellsCRITERIAOFAAEXCLUSIVEEVIDENCES:NoevidenceofmalignanciesNoinfitrationevidenceNomonoclonalevidenceNoevidenceofPNHNoevidenceofPNHRBCNodeficiencyofGPI-anchor-proteinNosoonerresponsetoprednisoneCRITERIAOFAAEXCLUSIVEEVIDENCES:NoevidenceofFANoFAfamilyhistoryNoFAchromosomefeatureNoevidenceofEvanssyndrome
&otherIRP(immunerelatedpancytopenia)Noauto-AbmediateddiseaseNoauto-AbsagainstRBC,BPC&BMCNoextravascularhemolysisNosoonerresponsetoprednisoneCRITERIAOFAAEXCLUSIVEEVIDENCES:NomalnutritionevidenceFolicacidand/orvitaminB12OthernutritionelementsNoendocrinediseaseevidenceNoevidenceoftemporaryfailureofhematopoiesisSUBTYPESOFAASAAPB:severepancytopeniaRC<15.0
109/L
NC<0.5
109/L
PC<20.0
109/LBM:widelyhypocellularIliac,SternalBMcellularity<25%SUBTYPESOFAAVSAA:OnekindofSAAPB:veryseverepancytopeniaNC<0.2
109/LOtherssameasthoseofSAABM:sameasSAANSAAAllAAcasesexceptSAAIn“CAA
(chronicAA)”categoryMortality/MorbidityPancytopenia:infectionandbleeding
Mortality>70%inSAAwithsupportivecarealone
BMTorimmunetherapy:drugtoxin,GVHD,otherclonaldiseases(MDS,PNH……)TREATMENTOFAATreatmentforsymptomsTreatmentforpathogenesisSYMPTOMATICTREATMENTOFAAOBJECTIVETorescuepatientsfromthefatalcomplicationsTomaintainpatient’slifeforrecoveryofhemopoiesisTocreatechancesforpathogenictreatmentSYMPTOMATICTREATMENTOFAATHERAPIESTherapiesofbleedingTherapiesofinfectionTherapiesofsevereanemiaTherapiesofothercomplicationsSYMPTOMATICTREATMENTOFAATHERAPIESOFBLEEDINGPlatelettransfusionEpinephrinelikehemostatsSupplementofcoagulationfactorsAnti-fibrinolysisOthersSYMPTOMATICTREATMENTOFAATHERAPIESOFINFECTIONByexperiences
BysensitivitytestsHGFs:G-CSF(300ug/d,H)IVIg:2.5g~5.0g/dOthersupportivetherapiesProphylactictherapiesSYMPTOMATICTREATMENTOFAATHERAPIESOFSEVEREANEMIARBCtrasfusionSAA:maintainHb70~80g/LNSAA:maintainHb50~60g/LAAwithsevereinfectionIronchelationtherapySYMPTOMATICTREATMENTOFAATHERAPIESOFOTHERCOMPLICATIONSHeartdisease:heartprotectionLiverdisease:lipidplusamino
acidkidneydisease:reducedCSADiabetes:reducedprednisoneDisbalanceofinnerenvironment:supplyingdifficientelementsTREATMENTFORPATHOGENESISOFAAIMMUNOSUPPRESSIONHEMOPOIETICSTIMULATINGSTEMCELLTRANSPLANTATION
IMMUNOSUPPRESSIONTHERAPIESOFAACORTICOSTEROIDSATG
(antithymocyteglobulin)/ALG(antilymphocyteglobulin)MONOCLONEAb:CD3CSAHD-IVIgCTX(cyclophosphamide)ATG/ALG+CSA(Immunosuppressivetherapy,IST)Immunosuppressivetherapy5yearsurvival:60-80%(Bacigalupoetal,2000)
Indication:1.Nomatchdonor2.SAA3.Age>35-40yearsoldImmunosuppressivetherapyATGLysislymphocyteCyclosporinBlockTcellfunctionATG+CSA
(betterthanATGorcyclosporinalone)Response>3monthsLong-termfollowupRelapse(10%)Laterclonaldisease:PNH,MDS,AMLHEMOPOIETICSTIMULATINGINAAAndrogenFolicAcid
andVitB12HGFsG-CSF
300ug/dGM-CSFsamedosageaboveEPO
(erythropoietin)6000ug/dIL11(interleukin-11),TPO
(thrombopoietin)?STEMCELLTRANSPLANTATIONBMTwithAidentical
twindonorALLO-BMTOtherSCTsinAAwerefewReasons:Age,costs,donorHLA-identicaltransplantation75~90%longtermcureGVHD:acute18%chronic26%Indication:1.SAAorVSAA2.Age<30~40yearsoldHLA:hi
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