Hemostasis Dr Elsye

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    HEMOSTASIS

    Dr ELSYE SOUVRIYANTI , MKes, SpA

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    Hemostasis

    Proses fisiologis penghentian perdarahan di tempatterjadinya injuri

    Faktor yang berperan :1) pembuluh darah2) trombosit3) proses koagulasi dan enzim fibrinolitik4) jaringan

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    Platelet activation

    1. Adhesion

    Vascular damage

    Contact with collagen

    (Endothelial damage)

    2. Loose aggregation

    3. Firm ggregation

    4. Fibrin formation

    5. Clot retraction

    ADP Release

    Thrombin formation

    on platelet plug surface

    Coagulation

    Platelet retraction

    Thrombin formation

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    Coagulation cascade, the traditional concept

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    H E M O P H I L I A

    CLINICAL PICTURE, TREATMENT

    AND ITS PROBLEMS

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    Introduction

    Hemophilia is the most common inheritedbleeding disorder.There are:

    Hemophilia A : deficiency of factor VIII Hemophilia B : deficiency of factor IX

    Both hemophilia A and B are inherited as

    X-linked recessive disordersSymptoms could occur since the patientbegin to crawl

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    Epidemiology

    Incidence: hemophilia A ( 85%) 1 : 5,000 10,000 males(or 1 : 10,000 of male l i fe bir th) hemophilia B ( 15%) 1 : 23,000 30,000 males(or 1 : 50,000 of male l i fe bir th)

    Approximately 70% had family history ofbleeding problems

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    Genetic

    Inherited as sex (X)-linked recessive

    Genes of factor VIII/IX are located onthe distal part of the long arm (q) of Xchromosome

    Female (women) are carriers

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    HemophiliaClinical Classification*

    Severe Moderate Mild

    Coagulation factor level < 1% 1 - 5% > 5%

    Bleeding spontaneous mi ld trauma moderate trauma

    Episodes 1-2 x/wk 1 x/mo none

    Hemarthrosis common occasionall y rare

    * H emophil ia A or B

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    Clinical manifestation

    Bleeding: usually deep (hematoma, hemarthrosis) spontaneous or following mild trauma

    Type: hemarthrosis hematoma intracranial hemorrhage

    hematuria epistaxis

    bleeding of the frenulum (baby)

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    Hemarthrosis

    Most common type of bleedingFrequently affected joints in this order :

    knee

    elbowankleshoulderwristhipetc.

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    8%

    25%

    44%

    15%

    5%

    lain-lain 3%

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    Carrier assessment

    A woman is an obligate carrier of hemophilia if one of the following condition is met:

    her father is a hemophiliac she has given birth to 2 or more hemophiliac sonsshe has given birth to 1 hemophiliac son and there is awell documented family history of hemophilia on thematernal side of her pedigree

    Confirmed by laboratory test:ratio F-VIII:C / F-VIII:Ag < 1chromosome analysis

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    1. Prompt interventionR est Ice C ompressionE levation

    2. Definitive treatment replacement therapy

    Treatment

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    Management of bleeding episode

    Stop bleeding with clotting factor within 2hours of onsetVeins should be well reservedAvoid the use of anti-aggregation drugsHome therapy

    Followed by comprehensive treatment

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    Comprehensive care

    To be governed through multidisciplineapproach involving experts in the field of:

    hematology occupational therapy

    orthopedics vocational therapyinfectious disease psychology

    nutrition nursing

    dentistry genetics

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    Blood component replacement therapy

    factor-VIII factor-IX (unit/ml) (ml)

    fresh-frozen plasma ~ 0,5 ~ 0,6 200

    cryoprecipitate ~ 4,0 - 20factor-VIII concentrate 25 - 100 - 10factor-IX concentrate - 25 - 35 20

    source of F-VIII: - monoclonal antibody purif ied;- intermediate- and high-purity;- recombinant

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    DosageF-VIII : 20 - 25 U/kgBW/12 hrs

    F-IX : 40 - 50 U/kgBW/24 hr

    Loading dose: 2 x

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    Dosage regulation:F-VIII demand = BW (kg) x F-VIII desired(%)

    (units) 1,5

    Desired F-VIII for:hemarthrosis 15%

    minor sur gery 30%major sur gery 60%

    intr acranial hemorrhage 100%

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    Other aim of treatment:

    DDAVP Genetic engineering Supportive: antifibrinolytic agent

    analgesics Physiotherapy (rehabilitation)

    Prophylaxis

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    Complications

    development of factor-VIII inhibitor(about 20%)treatment of inhibitor:

    increase dose of F-VIII

    shortcut coagulation pathwayuse of F-VIII from other species

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    Complications

    In the past there were several diseasestransmitted by transfusion such as:

    MalariaSyphilis

    HepatitisHIV/AIDS

    With adequate screening and storage theTTD can be diminished

    There are still risk of transmission with:Parvovirus B 19Creutzfeld-Jacob disease

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    Sports for hemophiliacs

    Permitted: Not permitted : swimming soccer

    table-tennis self defensetennis boxinggolf wrestling

    squash racingrowing hockey

    diving

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    Hemophilia Camp

    Teach self infusionEnhance interaction amongst patientsand parentsConduct seminars (medical or social)Evaluation of orthopedic status

    Discuss join activities (sports, etc.)

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    Hemophilia patients are as good as normalhuman resources in productivity, but because of

    expensive and limited source of F-VIII/F-IX

    could not optimally treated

    risk of being crippled/handicapped

    function

    productivity

    society burden

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    Purpura TrombositopenikImun

    CLINICAL PICTURE, TREATMENT

    AND ITS PROBLEMS

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    DEFINISI

    Purpura trombositopenia idiopatik padaanak adalah penyakit perdarahan akibat

    penghancuran trombosit yang berlebihan,dapat timbul dalam bentuk akut dan bersifatsementara, maupun rekuren yang disertaimasa remisi (kronis).

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    EPIDEMIOLOGI

    Anak > dewasaPerempuan : laki-laki 1 : 1

    Puncak usia 3-5 tahun Amerika serikat 100 tiap 1 juta orang/tahunIKA FKUI Juli 2001-Juni 2003 79 kasus

    perempuan : laki-laki 1,8 : 1

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    PATOGENESIS

    Antigen virus berikatan dengan Fab antibodi(kompleks imun)

    Beredar dalam sirkulasi

    Menempel pada permukaan trombosit

    Bagian Fc antibodi berikatan dengan reseptor Fcmakrofag sehingga terjadi fagositosis di limpa dan

    hati

    Faktor pencetus belum diketahui

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    MANIFESTASI KLINISPerdarahan Mendadak Perdarahan kulit

    petekie sampai ekimosissubkonjungtiva

    Perdarahan mukosa Epistaksis dan perdarahan gusi (30%), melena dan

    hematuri masif (5-10%)

    Perdarahan intrakranial1%,Trombosit < 10.000/uL, 4 minggu pertama sakit

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    MANIFESTASI KLINIS

    Dikutip dari Cines dkk 7 2002

    Dikutip dari Cines dkk7 2002

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    PEMERIKSAAN PENUNJANG

    Darah perifer lengkapHemoglobin normal atau sedikit

    menurunLekosit normal

    Trombosit < 100.000/uL

    Hapusan darah tepi : morfologi eritrositdan lekosit normal, trombosit normalatau sedikit lebih besar

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    Hapusan darah tepi

    Diunduh dari http://rds.yahoo.com/S=96062883/K=thrombocytopenia/v=2/SID=w/TID=I030_86/

    http://rds.yahoo.com/S=96062883/K=thrombocytopenia/v=2/SID=w/TID=I030_86/http://rds.yahoo.com/S=96062883/K=thrombocytopenia/v=2/SID=w/TID=I030_86/
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    PEMERIKSAAN PENUNJANG

    Aspirasi sumsum tulangKontroversi

    Tidak rutin untuk menegakkan diagnosisDilakukan bila gambaran hematologikhusus : sel imatur, netropenia persisten,

    anemia yang tidak dapat dijelaskan,morfologi eritrosit dan lekosit abnormal

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    PEMERIKSAAN PENUNJANG

    Antibodi antitrombositkurang sensitif dan kurang spesifik

    Anti HIV atau HIV DNAjika ditemukan faktor risiko

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    DIAGNOSIS

    AnamnesisPemeriksaan fisisDarah perifer lengkap yang sesuaidan konsisten dengan gambaran PTIakut

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    Tabel 1. Prinsip dasar anamnesis dan pemeriksaanfisis pada anak tersangka PTI

    Anamnesis Gejala perdarahan:

    Jenis, derajat dan lama perdarahan. Didahului tindakan invasif atau tidak Gejala sistemik:

    terutama yang terjadi dalam 6 minggu terakhir, infeksi virus seperti varicella,atau

    infeksi berulang seperti pada penyakit defisiensi imun Imunisasi virus hidup beberapa saat sebelum sakit Penggunaan obat-obatan seperti heparin, kinin, sulfonamide atau aspirin Faktor risiko HIV Riwayat trombositopenia atau kelainan darah dalam keluarga Riwayat trauma

    Pemeriksaan Fisis Gejala perdarahan (jenis dan derajat beratnya) Hati, limpa dan kelenjar getah bening Tanda infeksi Gambaran dismorfik yang menunjukkan kelainan kongenital Gambaran sindrom kongenital

    Dikutip dari George JN dkk 3

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    Tabel 2. Diagnosis banding trombositopenia akut

    Purpura trombositopenia akut

    DICSepsisSindrom hemolisis uremikLeukemia akutAnemia aplastikDikutip dari Buchanan GR 2

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    TATA LAKSANA

    Terapi ? kontroversi80-90% sembuh spontan, < 6 bln

    Tujuan terapi : menghentikan perdarahan meningkatkan jumlah trombosit sampai

    kadar aman

    mencegah perdarahan intrakranial

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    TATA LAKSANA

    American Society of Hematology 3 dan BritishPediatric Hematology Group 16

    Tr 30.000/uL, tanpa gejala/ringan (-)

    Tr < 20.000/uL, perdarahan mukosa bermaknaatau Tr < 10.000/uL, purpura ringan kortikosteroidatau imunoglobulin ivPerdarahan berat yang mengancam jiwa kortikosteroid dosis tinggi iv, imunoglobulin iv,transfusi trombosit

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    TATA LAKSANA

    British Pediatric Hematology Group 16Imunoglobulin G hanya pada :1. Terapi gawat darurat, tidak remisi dengankortikosteroid atau perdarahan aktif2. Persiapan operasi yang tidak dapat ditundaatau ekstraksi gigi darurat

    Fujisawa 17Gejala klinis ringan, trombosit > 10.000/uL tanpa terapi

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    PROGNOSIS

    Baik morbiditas dan mortalitas rendah

    80-90% sembuh spontan 10-20% menjadi PTI kronik

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