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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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