von willibrand diseases
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KOMPONEN 2 YANG BERPERAN PADA
HEMOSTASIS & FIBRINOLISIS .
1. PEMBULUH DARAH .
2. TROMBOSIT .
3. FAKTOR KOAGULASI .
4. FAKTOR FIBRINOLISIS .
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PEMBULUH DARAH .
1. Menghasilkan von Willibrand Faktormerangsang adhesi trombosit .
2. Menciptakan dan meningkatkan
koagulasi .3. Mengaktivasi fibrinolisis .
4. Lepasnya tromboplastin jaringan
jalur ekstrinsik teraktivasi .
5. Endothel berfungsi sbg sntitrombotik okperan plasminogen aktivator hancurtrombus .
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6. Berpengaruh thd fungsi AT III
( inhibitor trombin )
7.Mengandung trombomodulin(reseptor trombin) .- Trombomodulin + Trombin
Aktivasi Protein C
( menghambat koagulasi ) .
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1. PENYAKIT KEL. PEMBULUH DARAH
1. INHERETED :
1.1. Purpura Vascular .
1.2. Teleangiectasia hemorrhagic
Herediter
1.3. Penyakit Von Willibrand .
1.4. Connective Tissue Disrders .
( Ehlers-Danlos syndr. ) : heredtarycollagen abnormalities + purpura ok defectplatelet aggregation , hyperextensibilitysendi .
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Coagulation factor disorders
Inherited bleeding
disorders
Hemophilia A and B
vonWillebrands
disease
Other factor
deficiencies
Acquired bleeding
disorders
Liver disease
Vitamin K
deficiency/warfarin
overdose
DIC
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Platelet functional disorders
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Laboratory evaluation of
von Willebrand diseaseClassification Type 1 Partial quantitative deficiency
Type 2 Qualitative deficiency
Type 3 Total quantitative deficiency
Diagnostic tests:
vonWillebrand type
Assay 1 2 3vWF antigen Normal
vWF activity
Multimer analysis Normal Normal Absent
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INHERITED PLATELET DISORDERSDIAGNOSTIC STRATEGY (1)
HEMORRHAGIC SYNDROME
or SYSTEMATIC INVESTIGATION
before surgery
because personal or familial
past history of hemorrhage
Bleeding Time
Platelet Numeration : N or
HYPOTHESIS :
INHERITED
PLATELET
DISORDER
Specific
platelet
studies
HYPOTHESIS :
. WILLEBRAND
DISEASEconfirmation
tests+ APTT + APTT N
INITIAL
CLINICAL
SITUATION
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Platelet Aggregation Curves
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Von-Willebrand Disease:
Coagulation + PLT disorder:Congenital disorder
Deficiency of vWF molecule
Part of FVIII,
Mediates platelet adhesion
Prolonged Bleeding timeLow Factor VIII & long aPTT
Mucocutaneous bleeding
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INHERITED PLATELET DISORDERS
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INHERITED PLATELET DISORDERSMAIN CHARACTERISTICS
PLATELET
FUNCTION DISORDER BT NUMBER
PLATELETS
MORPHOLOGY
SIZE - VOLUME
CLOT
RETRACTION
AGGREGATION Glanzmann Thrombasthenia
GPIIbIIIa or
N N O or
ADHESION Bernard Soulier S ndrome
GPIb IX V
others
* latelet seudo Willebrand
GPIb
* colla en reactivit
GPIa
or
N
N
Size
iant latelets
Size
Size
N
N
N
SECRETION
adhesion
a re ation
Stora e Pool Disease
ranules or N N or Size N N
Gra latelet S ndrome ranules
N or Nranules
N
ACTIVATION
release
a re ation
Ca++
fluxes abn.
Prosta landins s nthesis
decreased
* COX
* TX s nthase
N N ?
COAGULANTACTIVITIES
Platelet F.3 N N N ?
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Treatment of von Willebrand disease
Varies by Classification
Cryoprecipitate Source of fibrinogen, factor VIII and VWF
Only plasma fraction that consistently contains VWFmultimers
Correction of bleeding time is variable
DDAVP (Deamino-8-arginine vasopressin) Increases plasma VWF levels by stimulating secretion from
endothelium
Duration of response is variable
Used for type 1 disease
Dosage 0.3 g/kg q 12 hr IV
Factor VIII concentrate (Humate-P) Virally inactivated product
Used for type 2 and 3
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