內(nèi)科學(xué)課件 10、再障aplastic anemia 2015

APLASTICANEMIA

(AA)再生障礙性貧血

JCDENGMedicaltermsAnemia貧血Aplasticanemia再生障礙性貧血Pancytopenia

全血細胞減少Hypocellular

細胞減少的；低增生性的Neutrophil

中性粒細胞Lymphocyte淋巴細胞Platelet血小板Hemato-血液（前綴）.Hematopoiesisofbonemarrow

骨髓造血造血干細胞前體細胞

hematopoieticstemcellprogenitorcellseachlineageRBC

WBC

PLTResultsofbonemarrowfailurehematopoieticstemcellprogenitorcellseachlineageRBCanemia

WBCinfection

PLTbleedingDefinition

Aplasticanemiaisbonemarrowfailuresyndromes,characterizedbyhypocellularbonemarrowandpancytopeniainperipheralblood.Themajorsymptomsareanemia,infectionandbleeding.Morbidity發(fā)病率2to6peoplepermillionannually,ObviousprevalenceintheOrientChina:0.74/100,000

Male:female:nodifference

Classification1.AccordingtothecausesInheritedAA(遺傳性AA,Fanconianemia);AcquiredAA(獲得性AA）

primaryAA;secondaryAA2.Accordingtotheseverityofanemia

SevereAA(重型，SAA);NonsevereAA(非重型，NSAA)

·virusinfection(病毒感染)HBV;HCV------hepatitisvirusassociatedAA

parvovirusB19(細小病毒）-------pureredcellAA

Etiology病因?qū)W

Etiology·Virusinfection·Chemicalfactors

antibiotics:chloromycetin

(氯霉素）

SMZ(磺胺類）

antitumoragentsBenzene（苯）FromWikipedia（維基百科）Benezene：

anorganicchemicalcompound…animportantcomponentofgasoline…anotoriouscauseofbonemarrowfailure...

Etiology·Virusinfection·Chemicalfactors·exposuretoradiationx-ray;radioactiveisotopePathogenesis發(fā)病機制·

Stemcellinjury·Abnormalhematopoieticmicroenvironment·AbnormalimmunityStemcellinjury

bonemarrow:CD34+cellsfallX-Ray

StemCell

Micro-environmentAbnormalhematopoieticmicroenvironmentAbnormalimmunityTcellfunctionincreasedCD4+Tcell,CD8+TcellIL-2,IFN,TNF

majorpathogenesisThreetheoriesStemcellinjury-----SeedtheoryAbnormalhematopoieticmicroenvironment---SoiltheoryAbnormalimmunity----Insecttheory

Clinicalmanifestations·Generalpresentations:

anemia,bleedingandinfection,noobviousswellingofliver,spleenandlymphoidnodes·

ThedifferentmanifestationsofSAAandNSAA

onsetacute,quicklychronic,slowlyanemiamildatfirst,severe,aggravatedgraduallymajorsymptombleedingsevere,GIbleeding,mild,petechiae

瘀點,brainbleeding,fatalpurpura

紫癜

infectionsevere,septicemia敗血癥

mild,fluprognosis預(yù)后

bad,highmortality死亡率

nottoobad

SAANSAA

Laboratoryexamination·PeripheralbloodRoutinecount

pancytopenia,Reticulocytes

網(wǎng)織紅細胞

SAANSAA

neutrophil<0.5×109/L>0.5×109/LPLT<20×109/L>20×109/L

reticulocyte<15×109/L>15×109/L

Laboratoryexamination·Bonemarrowaspiration骨髓穿刺

SAA:severelyhypocellularinmultiplecites,nomegakaryocyte

巨核細胞,non-hematopoieticcellsareactive

NSAA:hypocellularinmultiplecites,

megakaryoctyesdecreased

Bonemarrowsmear

Laboratoryexamination·

Bonemarrowbiopsy

骨髓活檢

Hematopoietictissuedecreasedequably;FattissueincreasedSAANSAA

Diagnosis

HistoryClinicfeaturesPhysicalexaminationLaboratorydataDifferentialDiagnosis

pancytopenia·PNH陣發(fā)性睡眠性血紅蛋白尿·MDS骨髓增生異常綜合征·IAP自身抗體介導(dǎo)的全血細胞減少·Acuteleukemia急性白血病……

Treatment·SupportivetreatmentProtectivemanagementCorrectanemiaControlbleedingControlinfectionLaminarairflow

room

Protectivemanagementpreventinfection;food,environment,dentalhygiene口腔衛(wèi)生CorrectanemiaBloodtransfusionPackedRBC濃縮紅細胞

,Hb<60g/LControlbleedingHemostatic

止血劑PlatelettransfusionPLT<20×109/LControlinfection·blood,secretionculture·broadspectrumantibiotics:

bacteralinfection:thirdgenerationcephalosporin（頭孢）,Imipenem(泰能）,fungusinfection:amphotericinB

(兩性霉素B)·

Morrowgrowthfactors:G-CSF,GM-CSFTreatment·TherapyofSAA1.BoneMarrowTransplantation

allogeneicBMT(異基因造血干細胞移植）Female,20PancytopeniaBonemarrowaspirate:dilute;drytapBonemarrowbiopsy:hypocellularTcellFCM:nosi