8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
1/54
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
2/54
What isWhat is haemostasishaemostasis??
A body mechanism to stop traumatic bleeding spontaneousA body mechanism to stop traumatic bleeding spontaneousand localized, and to keep the fluidity of blood in vascular.and localized, and to keep the fluidity of blood in vascular.
BalanceBalance haemostasishaemostasis require:require: Bloodvessel
orma oo vesse sorma oo vesse s
Normal plateletsNormal platelets
Normal coagulation factorsNormal coagulation factors
Inhibitors Platelet
ormaorma r no ys sr no ys s an o eran o er
regulating proteins/inhibitorsregulating proteins/inhibitorsHemostasis
FibrinolysisCoagulation
factors
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
3/54
HemostasisMx NORMAL HEMOSTASIS & KOAGULASI:
1. Spasme dinding pembuluh darah (fase vaskularmis: Carbazochrome
2. Peranan fungsi platelet (fase platelet)
3. Faktor Koagulasi (fase koagulasi)
mis: Vit. K
. s em an oagu an an an r no s s
mis: Asam traneksamat ( anti fibrinolisis )
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
4/54
Hemostasis
1. Vascular Phase - Vasoconstriction
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
5/54
Senile PurpuraSenile Purpura
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
6/54
Petechiae inPetechiae in
VasculitisVasculitis
(Rocky Mountain Spotted Fever)(Rocky Mountain Spotted Fever)
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
7/54
-- ImmuneImmune
disorderdisorder ChildrenChildren
FollowsFollows
infectioninfection PetechiaePetechiae withwith
edema andedema and
itching.itching.
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
8/54
--
20y Male, fever, painful symmetric polyarthritis for a day. During the next two days, edema and palpable purpura developed.
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
9/54
2. Platelet Phase ( Platelet Plug )
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
10/54
ROMBOSITROMBOSITJumlahnya adalah 150 450 x 10 / mmDiameter 2.5
Fungsi : adhesi, sekresi, agregasi pembentukan
Diproduksi di sumsum tulang melalui fragmentasisitoplasma
megakariosit (1 megakariosit 4000 trombosit)Produksi trombosit dikendalikan oleh mekanisme
umora ya u ormon rom opo e n
Trombopoietin disintesis oleh hati (>>>) dan ginjal
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
11/54
--
PetechiaePetechiae && PurpuraPurpura
spontaneous bleeding afterspontaneous bleeding after
CNS bleeding (CNS bleeding (severesevere pltplt))
Prolonged bleeding time (BT)Prolonged bleeding time (BT)
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
12/54
Platelet count < 100,000/LPlatelet count < 100,000/L
Spontaneous bleeding if < 20,000 /LSpontaneous bleeding if < 20,000 /L
If BMP shows increased ofIf BMP shows increased of megakaryocytesmegakaryocytes
If vice versa means disorders of plateletIf vice versa means disorders of platelet
PseudothrombocytopeniaPseudothrombocytopenia: platelet clumping: platelet clumpingsecondary to collection of blood in EDTAsecondary to collection of blood in EDTA
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
13/54
..
marrow injury (drug, irradiation), marrowmarrow injury (drug, irradiation), marrow, , ,, , ,
failure (failure (e.ge.g aplasticaplastic anemia)anemia)
2. Sequestration due to2. Sequestration due to splenomegalysplenomegaly
3. Accelerated destruction3. Accelerated destruction
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
14/54
Causes of Accelerated DestructionCauses of Accelerated Destruction Drugs:Drugs:
ChemoChemo txtx,, thiazidethiazide, ethanol, estrogen, sulfonamides,, ethanol, estrogen, sulfonamides,
quinidinequinidine, quinine, methyldopa, quinine, methyldopa Heparin Induced ThrombocytopeniaHeparin Induced Thrombocytopenia
Autoimmune DestructionAutoimmune Destruction
Idiopathic ThrombocytopenicIdiopathic Thrombocytopenic PurpuraPurpura ( ITP )( ITP )
Thrombotic ThrombocytopenicThrombotic Thrombocytopenic PurpuraPurpura ( TTP )( TTP )
Hemorrhage with extensive transfusionHemorrhage with extensive transfusion
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
15/54
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
16/54
PlateletPlatelet dysfunctiondysfunction
n er e sor ers:n er e sor ers:
BernardBernard--SoulierSoulier diseasedisease
large platelets, failure oflarge platelets, failure of adhesionadhesion
GlanzmannsGlanzmanns thrombastheniathrombasthenia normal size, failure ofnormal size, failure of aggregationaggregation
DrugsDrugs -- Aspirin, Alcohol, Uremia,Aspirin, Alcohol, Uremia,
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
17/54
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
18/54
Platelet deficiency..
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
19/54
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
20/54
3. Blood Clot
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
21/54
PEMBEKUAN DARAHPEMBEKUAN DARAH(KOAGULASI)(KOAGULASI)
Meru akan sistem am lifikasi biolo isMeru akan sistem am lifikasi biolo is
ProkoagulanProkoagulan aktivasi oleh proteolisisaktivasi oleh proteolisis faktor koagulasifaktor koagulasiaktif (bersifat enzim)aktif (bersifat enzim) mengaktifkan prokoagulanmengaktifkan prokoagulan
er u nyaer u nya
CASCADE KOAGULASICASCADE KOAGULASI
em en u an r nem en u an r n
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
22/54
NomorNomor NamaNama
II FibrinogenFibrinogenSemua faktor koagulasi mrpk
IIIIII TromboplastinTromboplastin / Tissue Factor/ Tissue Factor
IVIV CalciumCalcium
Semua enzim mrpk serine protease
a e ac ora e ac or roacce er nroacce er n
VIIVII ProconvertinProconvertin / Serum/ Serum ProthrombinProthrombinConversion Accelerator (SPCA ) / StableConversion Accelerator (SPCA ) / Stable
kecuali Faktor XIII
VIIIVIII AHF (AntiAHF (Anti HemofilicHemofilic Factor)Factor)
IXIX PlasmaPlasma ThromboplastinThromboplastin ComponentComponent
(Faktor Jaringan) terdapat pada
permukaan jar perivaskuler,
berikatan dengan Faktor VII (TF-
XX StuartStuart ProwerPrower FactorFactor
XIXI PTA (PlasmaPTA (Plasma ThromboplastinThromboplastin
FVII)
Istilah faktor VI tdk lg digunakan,
n ece enn ece enXIIXII Hageman Factor (Contact Factor)Hageman Factor (Contact Factor)
XIIIXIII Fibrin Stabilizing FactorFibrin Stabilizing Factor
sebab apa yang semula dianggapfaktor VI yg terpisah, sekarangdiketahui adalah bentuk aktif
-- PrekallikreinPrekallikrein (Fletcher Factor)(Fletcher Factor)
-- HMWK (High Molecular WeightHMWK (High Molecular WeightKininogenKininogen))
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
23/54
Blood Coagulation & TestsBlood Coagulation & Tests
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
24/54
Inisiator koagulasi
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
25/54
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
26/54
..misalnyamisalnya dengandengan obatobat asamasam tranexamattranexamat
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
27/54
.. rnoyssrnoyss
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
28/54
Fibrinol sisFibrinol sis Normal body process to breakdown fibrin/clotsNormal body process to breakdown fibrin/clots
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
29/54
PG
t-PAt-PA
PG
PlasminogenPlasminogen
PlasminPlasmin
-
PG PL
-
PG PLFDPsFDPs
FibrinFibrinFibrin
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
30/54
Faktor XIa, XIIa,HMWK, Kallikrein
tPA (tissue plasminogenActivator), Urokinase
Plasminogen Plasmin
Fibrin / Fibrinogen Fragmen X
Fragmen Y + D
Fragmen E + D
Sistem FibrinolitikSistem Fibrinolitik
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
31/54
Primary and SecondaryPrimary and Secondary HemostasisHemostasisInjury to vessel wall ( exposure of subendothelium )
Adhesion of platelets
Vasoconstriction
Activation and aggregation of platelets
Primary hemostatic thrombus (white thrombus)
Activation of coagulation cascade
Activation of thrombin and formation of fibrin
Intrinsic (F XII/F XI) and extrinsic pathways (tissue factor)
Secondary hemostatic thrombus (red thrombus)
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
32/54
Coagulation & fibrinolysis should be in balanceCoagulation & fibrinolysis should be in balance
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
33/54
IMBALANCESIMBALANCES
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
34/54
HaemostasisHaemostasis estingsestings
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
35/54
HaemostasisHaemostasis TestingsTestings Abnormalities are notnormall detectable b
laboratory tests
vessel
, ,
plateletaggregometry
Inhibitors Platelet
Hemostasis
Coagulation
PT, APTT, TT, vWF, coagulation factors,
Protein C, Protein S, AT-III, LA1, LA2, D-Dimer
C diti fC diti fC diti fC diti f
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
36/54
Conditions ofConditions ofConditions ofConditions of
coagu opat ycoagu opat ycoagu opat ycoagu opat y
Hemophilia Liver disease
DIC
Anticoagulant treatment
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
37/54
OnsetOnset -- delayed after traumadelayed after trauma
Deep bleedingDeep bleeding
--
Into deep tissuesInto deep tissues HematomaHematoma
arge s n eearge s n ee cc ymosescc ymoses
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
38/54
Acute bleedingAcute bleeding
Diseases with potentialDiseases with potential hemostasishemostasis disorders: liverdisorders: liver
disease se sis DICdisease se sis DIC thrombo hiliathrombo hilia.. Screening preScreening pre--operative/invasiveoperative/invasive
Patient that are havin or will have anticoa ulantPatient that are havin or will have anticoa ulant
therapytherapy
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
39/54
Platelet countPlatelet count
Activated Partial Thromboplastin timeActivated Partial Thromboplastin time
Thrombin Time (TT)Thrombin Time (TT)
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
40/54
PTPT APTTAPTT DeficiencyDeficiency
NN>>
>>NN
XII, XI, IX, VIIIXII, XI, IX, VIIIVIIVII
>> >> X, V, II, IX, V, II, I
Mixing Study:To distinguish deficiency from inhibitor
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
41/54
Normal bleeding time & Platelet countNormal bleeding time & Platelet count
ProlongedProlonged prothrombinprothrombin time (PT)time (PT)
deficiencies of II, V, VII, Xdeficiencies of II, V, VII, X Prolonged time (Prolonged time (aPTTaPTT))
all factors exce t VII XIIIall factors exce t VII XIII
Mixing studiesMixing studies -- normal plasma correctsnormal plasma corrects
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
42/54
Classic hemo hilia hemo hilia AClassic hemo hilia hemo hilia A
XX--linkedlinked receciverececive (affects 1 males)(affects 1 males) Most commonMost common -- severe bleedinsevere bleedin
Spontaneous hematomasSpontaneous hematomas < 1, 5, 75%< 1, 5, 75%
AbnormalAbnormal aPTTaPTT Intrinsic path.Intrinsic path. Diagnosis:Diagnosis:
Immunoassay: factorImmunoassay: factor VIIIVIII ( N: 52( N: 52--100% )100% )
Po ymorp c DNA pro esPo ymorp c DNA pro es TreatmentTreatment -- factor VIIIfactor VIII concentrate, Cryoprecipitateconcentrate, Cryoprecipitate
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
43/54
Factor IX DeficiencyFactor IX Deficiency
Christmas disease (Hemophilia B)Christmas disease (Hemophilia B)
--
Indistinguishable from classic hemophilia (FIndistinguishable from classic hemophilia (FVIIIVIII
AbnormalAbnormal aPTTaPTT Intrinsic pathIntrinsic path
Dia nosis:Dia nosis: factor IXfactor IX ( N: 52( N: 52--100% )100% )
Re uires evaluation of factor VIII and IXRe uires evaluation of factor VIII and IX
activity levels to diagnoseactivity levels to diagnose TreatmentTreatment -- factor IX concentratefactor IX concentrate
Cryoprecipitate if factor IX unavailableCryoprecipitate if factor IX unavailable
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
44/54
VonVon WillebrandWillebrand DiseaseDisease
PenyakitPenyakit perdarahanperdarahan kongenitalkongenital (( autosomalautosomal dominantdominant
melekatkanmelekatkan trombosittrombosit keke endotelendotel ,,
diperlukandiperlukan pdpd adesiadesi trombosittrombosit && berperanberperan sbgsbg
pembawapembawa fcfc VIIIVIII DefisiensiDefisiensi//abnormalitasabnormalitas fcfc VWVW tjdtjd abNabN hemostasishemostasis
primerprimer
RiwRiw PenyakitPenyakit:: EpistaksisEpistaksis,, ptekieptekie,, hematomhematom,, menoragimenoragi,, perdarahanperdarahan gusigusi //
salsal.. CernaCerna (( spontanspontan // tdktdk ))
TandaTanda && GxGx KlinikKlinik::
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
45/54
TandaTanda && GxGx KlinikKlinik::
Rin anRin an:: erdarahanerdarahan mukokutanmukokutan GIBGIB e istaksise istaksismenoragimenoragi ))
BeratBerat hemartrosishemartrosis,, hematomhematom intraintra muskularmuskular
Lab: BTLab: BT , APTT N/ , F VIII ,, APTT N/ , F VIII , vWFvWF N/N/
TipeTipe I (70%) :I (70%) : defekdefek kuantitatifkuantitatif DDAVPDDAVP
pepe :: e ee e ua aua a onsen raonsen ra
F VIII (F VIII ( tergantungtergantung tipetipe ))
Platelet type (pseudoPlatelet type (pseudo--VWD) :VWD) : defekdefek glikoproteinglikoprotein IbIb
Cryoprecipitate ( f VIII, fibrinogen,Cryoprecipitate ( f VIII, fibrinogen, vWFvWF ))
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
46/54
Acquired coagulation disorderAcquired coagulation disorder::
Vitamin K deficiencyVitamin K deficiency
-- neonatesneonates -- decreased intestinaldecreased intestinal
flora and dietary intakeflora and dietary intake-- oral anticoagulants (oral anticoagulants (coumadincoumadin))
-- fatfat malabsorptionmalabsorption syndromessyndromes
, , ,, , ,
Prolonged PT andProlonged PT and aPTTaPTT
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
47/54
Combined Primar and SecondarCombined Primar and SecondarHemostaticHemostatic DisordersDisorders
..
CoagulationCoagulation Major pathologic processesMajor pathologic processes --
obstetric complications,obstetric complications, neoplasmsneoplasms,,
n ec on seps s , ma or rauman ec on seps s , ma or rauma
PrimaryPrimary -- platelet consumptionplatelet consumption
ee ng me,ee ng me, p a e e sp a e e s SecondarySecondary -- factor consumptionfactor consumption
,,
DDi i t di i t d II t lt l CC l til ti
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
48/54
DDisseminatedisseminated IIntravascularntravascular CCoagulationoagulation
A complex systemic thrombohemorrhagic disorder involving the generation ofintravascular fibrin and the consumption of procoagulants and platelets. The resultantclinical condition is characterized by intravascular coagulation and hemorrhage.
Common Causes of DICCommon Causes of DIC
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
49/54
Common Causes of DICCommon Causes of DICInfections*Infections*
-- , ,, ,EnterobacteriaceaeEnterobacteriaceae GramGram--positive bacteria:positive bacteria: PneumococcusPneumococcus, Staphylococcus, Hemolytic Streptococcus, Staphylococcus, Hemolytic Streptococcus Viral: Hepatitis,Viral: Hepatitis, varicellavaricella, cytomegalovirus, hemolytic streptococcus, cytomegalovirus, hemolytic streptococcus
CancerCancer--related*related*
Leukemia: AcuteLeukemia: Acute promyelocyticpromyelocytic, acute, acute myelogenousmyelogenous, chronic, chronic myelogenousmyelogenous, acute, acutelymphoblasticlymphoblastic Solid tumors: Lun breast stomach rostate ancreas ovarianSolid tumors: Lun breast stomach rostate ancreas ovarian biliarbiliar tracttract Blood transfusion reactions: Acute hemolytic transfusion reactions, massiveBlood transfusion reactions: Acute hemolytic transfusion reactions, massive
transfusion of whole bloodtransfusion of whole blood Liver disease: Liver failure secondary to primary liver disease or metastasisLiver disease: Liver failure secondary to primary liver disease or metastasis
Prosthetic devices:Prosthetic devices: PeritoneovenousPeritoneovenous shuntsshuntsObstetric complicationsObstetric complications
Amniotic fluid embolismAmniotic fluid embolism Premature separation of placentaPremature separation of placenta EclampsiaEclampsia; retained placenta; retained placenta
Septic a ortionSeptic a ortion
Widespread tissue damageWidespread tissue damage Following surgery or traumaFollowing surgery or trauma After severe burnsAfter severe burns
Vascu ar a norma itiesVascu ar a norma ities KasabachKasabach Merritt syndromeMerritt syndrome
Leaking prosthetic valvesLeaking prosthetic valves Cardiac bypass surgeryCardiac bypass surgery
Test Res lts in DICTest Res lts in DIC
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
50/54
Test Results in DICTest Results in DICenera Testsenera Tests
Platelet count: DecreasedPlatelet count: Decreased Fibrino en level: DecreasedFibrino en level: Decreased Peripheral blood smear:Peripheral blood smear: SchistocytesSchistocytes DD--dimerdimer assay: Elevatedassay: Elevated
Tests to Determine Accelerated CoagulationTests to Determine Accelerated Coagulationrr : r: r FibrinopeptideFibrinopeptide A: ElevatedA: Elevated ProthrombinProthrombin activation peptides (F1 & F2): Elevatedactivation peptides (F1 & F2): Elevated
hrombinhrombin--antithrombinantithrombin complexes: Elevatedcomplexes: Elevated
PlasminogenPlasminogen: Decreased: Decreased
AlphaAlpha--22--antiplasmin: Decreasedantiplasmin: Decreased
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
51/54
..
PrimaryPrimary -- dysfunctional platelets and/ordysfunctional platelets and/or
thrombocytopenia (thrombocytopenia ( BT)BT)
SecondarySecondary -- decrease in all coagulationdecrease in all coagulation
factors exce tfactors exce t vWFvWF PTPT aPTTaPTT Vitamin K will promote synthesis ofVitamin K will promote synthesis of
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
52/54
BTBT PltPlt PTPT PTTPTT
Vascular DisVascular Dis -- -- -- --
PLT DisorderPLT Disorder -- -- -- --
*Congenital*Congenital -- -- --
Vit K / LiverVit K / Liver*Acquired*Acquired -- -- --
Combined (DIC)Combined (DIC) --
SummarySummary
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
53/54
SummarySummary
Symptom Platelet Coagulation
Petechiae Yes No
ites kin &
Mucosa
Deep Tissue
Time Immediate Delayed
cc ymoses/Hematomas es es
8/11/2019 Hemostasis & Coagulation Disorders(Ringkas II) - Dicky [Compatibility Mode]
54/54
Top Related