Hemostasis Dr Elsye
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Transcript of 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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