DisordersofHemostasis(stopbleeding)

PlateletAggregation Clotting

1.Vasoconstriction
2.Plateletadhesion
3.Plateletdegranulation
4.Plateletaggregation
INTRINSIC=TENETTwelve,eleven,nine,eightTen

EXTRINSIC=7

FibrinogenThrombin>fibrincrosslinkedforStable
plateletfibrinThrombus

Allclottingfactorsproducedbyliver
Endresult? WeakPLATELETplug(weakb/cplateletsconnectedw/
fibrinogenlink,notfibrin)
FibrincanbecrosslinkedSTABLEplateletfibrin
THROMBUS
Clinical
Featuresw/
disorders
1)Mucosalbleeding=epistaxis(nosebleedingmost
common),hemoptysis,menorrhagia,bleedafterdental

2)Skinbleeding=petechiae(12mm),purpura(>3mm),
ecchymoses(>1cm),easybrusing
1)Hemoarthrosis:Deeptissuebleedingintomuscles&
joints

2)Riskoflifethreateninghemorrhage:esp.headtrauma
cerebralhemorrhage
Coagulation
Studies
1.PlateletCount(quantitative)nl:150400K/ul=<50K/ul

2.bleedingtimenl27mins=prolonged
1.PTT=intrinsic&commonfactors=iffactorsPTT
(timemeasure)

2.PT=extrinsic&commonfactors=iffactsPT(time
measure)

Morenumbers,moreletteredtest
Coagulationstudies:Plateletcount,bleedingtime,PT,PTT

ClottingDisorders
PP Management PlateletCount Bleedingtime PT PTT
CONGENITAL
HemophiliaA F8deficiency
Xlinked
giveF8 Nl Nl Nl High
VonWilliebrandDisease vWFsecondaryF8(vWFbindsF8)

rangefrommilddeficiencytocompleteabsenceofvWF

Note:vWFneededfor1o,F8for2o
Milddef:
giveDDAVP(inducesvWFreleasefrom
endothelialcells)

Severedef:
Cryoprecipitate(containsvWF)
Nl High Nl High
HemophiliaB F9deficiency giveF9 Nl Nl Nl High
Xlinked
ACQUIRED
DIC(typeofMHA) 2otosepsis,malignancy,largehemangiomas

useupeverything:
Platelets
Allclottingfactors(including5,8)
fibrinogen(w/elevatedfibrindegradationproducts)
treatunderlyingcause

fribinogen,FFP,plateletsPRN
Low High High High
VitaminKdef. LoseVitKdependentfactors:
2,7,9,10&ProteinC&SNOT5or8

CP:hemorrhagicdiseaseofnewborn
VitaminKIMafterbirth Nl Nl High High
Liverfailure 2,7,9,10,&5NOT8 VitaminK,FFP,plateletsPRN Nl Nl High High

PlateletDisorders
Management PlateletCount Bleeding
time
PT PTT
QUANTITATIVE=
THROMBOCYTOPENIA

ImmuneThrombocytopenicPurpura
(ITP)
IgGbinds,spleenicmacrophageeatsit IVIGthrowstickatdog Low High Nl Nl
MicroangiopathicHemolyticAnemia Microangiopathic=microthrombi=platelet
Hemolytic=RBC

HemolyticUremicsyndrome(HUS):EHECshigalike
toxin

Thromboticthrombocytopenicpurpura(TTP):low
ADAMSTThighvWF

HeparinInducedThrombocytopenia(HIT):Heparinbinds
toplatelet(formshapten)thenIgMbindsanddestroys
treatunderlyingcause Low High Nl Nl
QUALITATIVE
GrayPlatelet

BernardSoulier

Glanzmann
Greyisadog,bernardisblackgetsbs,glanzmannis
jewishgetsaandbs

Gray=agranulevWF
BS=g1bradhesionfucked
Glanzman=gp2b/3aaggregationfucked
Nl High Nl Nl