diatesis hemoragis (kulia 4 ipd iii).ppt
TRANSCRIPT
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HEMOSTASIS - DIATESIS HEMORAGIS
- TROMBOSIS
Vaskular
Trombosit Koagulasi
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A. VASKULAR* Vasokonstriksi* Aktifasi trombosit* Aktifasi faktor Koagulasi
B. TROMBOSIT* Adesi* Agregasi* RX pelepasan isi trombosit Granula padat : ADP, ATP, Ca, Epinefrin, Norepinefrin, Granula alfa : Fibrinogen, vWF, FV, PF 4, TG, Lisosom : Enzim asam hidrolase
C. SISTIM KOAGULASI VS FIBRINOLISIS
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NOMENCLATUR FAKTOR PEMBEKUAN DARAH
I FibrinogenII ProtrombinIII Tissue factorIV Ion calsiumV ProaccelerinVI -VII ProconvertinVIII Anti hemophilic factorIX Plasma tromboplastin componentX Stuart factorXI Plasma tromboplastin antecedentXII Hageman factorXIII Fibrin stabilizing factor - High moleculer weight kininogen - Pre kalikrein
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Jalur Intrinsik Jalur Ekstrinsik
XII VIIKontak Ca Tromboplastin
Jaringan XIIa
HMWK XI XIa
IX IXa VIIa PF3, VIII, Ca
X Xa V, PF3, Ca
Fibrinogen Protrombin Trombin
Fibrin monomer
Fibrin polimer Solubel
XIII XIIIa
Ca Fibrin polimer InSolubel
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Intrinsik Extrinsik Eksogen
XIIa, Kalikrein t-PA Urokinase Aktifator Plasminogen
Plasminogen terikat Plasmin terikat Fibrin
FDP
Plasminogen bebas Plasmin bebas FibrinogenFc V, Fc VIII
Anti Plasmin
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PEMERIKSAAN PENYARING KELAINAN HEMOSTASIS1. RUMPEL LEEDE TEST
tu ketahanan ddg kapiler, tetapi juga fgs / jml trombosit
2. BLEEDING TIME Normal 1 – 6 menit tu extra vasc, tetapi juga ddg kapiler & trombosist
4. TROMBOSIT Normal 150000 – 400000 / mm3
3. CLOTHING TIME Normal 1 – 4 menit
5. PROTROMBIN TIME (PT)(11 – 15 ‘)INR menguji antikoagulan oral Jalur ekstrinsik, Fc X, Fc V, protrombin, fibrinogen
6. ACTIVATED PARTIAL THROMBOPLASTIN TIME ( APTT ) ( 20 – 40 ‘) Jalur intrinsik, Fc X, Fc V, Protrombin, fibrinogen Heparin 1,5 – 2,5 x normal
7. TROMBIN TIME (16 – 20’) menguji fibrinogen, heparin, FDP
8. PEMERIKSAAN Fc XIII
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Kelainan hemostasis Primer Vaskuler & Trombosit Sekunder Fc Koagulasi
PENDEKATAN KLINIS KELAINAN HEMOSTASIS Anamnesis
* Sejak kanak-kanak Hemofili* Perdarahan masif saat pemotongan tali pusat Deff Fc XIII
Afibrinogenemia Deff Fc VII
* Delayed bleeding extr. Gigi* Perdarahan trauma / operasi / sirkumsisi
Pemeriksaan* Ptekia* Purpura* Ekimosis* Hematoma* Hamartrosis* Epistaksis, Perdarahan gusi, hemoptisis, hematemesis, melena, hematuria, metroragia
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TROMBOSITOPENI
PRODUKSI KONSUMSI
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Disorders with increased platelet consumption
Disorders with immune mechanism Autoimmune–idiopathic thrombocytopenic purpura
Alloimmune–post transfusion purpura, neonatal alloimmune thrombocytopenia
Infection-associated–infectious mononucleosis, HIV, malaria Drug-induced–heparin, penicillin, quinine, sulphonamides,
rifampicin
Thrombotic thrombocytopenic purpura/haemolytic uraemic Syndr.
Hypersplenism and splenomegaly
Disseminated intravascular coagulation
Massive transfusion
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Acquired disorders of reduced platelet production*
Drug induced Leukaemia
Metastatic tumour Aplastic anaemia Myelodysplasia Cytotoxic drugs Radiotherapy
Associated with infection Megaloblastic anaemia
*Due to bone marrow failure or replacement
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Idiopathic thrombocytopenic purpura ( ITP ) (Immune thrombocytopenic purpura )
• ITP AKUT- Anak / dewasa muda- Predileksi sex (-)- Riwayat infeksi virus ( 1-3 mg )- Perdarahan akut- Onset 2 – 6 minggu ( remisi
spontan pada 80 % kasus
• ITP KRONIK- Wanita muda pertengahan- Jarang riwayat infeksi
sebelumnya- Perdarahan menyusup- Onset bulan – tahun- Jarang remisi spontan
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DIAGNOSIS
ANAMNESIS
PEMERIKSAAN FISIK
LABORATORIUM * DARAH RUTIN
* FAAL HEMOSTATIK* BMP
* PETANDA IMUN
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PENATALAKSANAAN
- TRANSFUSI TROMBOSIT - HINDARI TRAUMA / DRUG INDUCED- KORTIKOSTEROID- IMUNOGLOBULIN
BILA REFRAKTER - IMUNOSUPRESIF
SIKLOFOSPAMID, AZATIOPRIN, VINKRISTIN- SPLENEKTOMI
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HEMOFILIHEMOFILI A : DEFISIENSI FC VIIIHEMOFILI B : DEFISIENSI FC IX
* HEREDITER, X LINKED RESESIF
* MANIFESTASI PERDARAHAN : TGT KADAR FC VIII - 50 – 100 % PERDARAHAN (-)- 25 – 50 % PERDARAHAN SETELAH TRAUMA BESAR- 5 – 25 % PERDARAHAN SETELAH TRAUMA KECIL
PERDARAHAN SPONTAN (-)- 1 – 5 % PERDARAHAN SETELAH TRAUMA KECIL,
KADANG DENGAN PERDARAHAN SPONTAN- 0 % PERDARAHAN SPONTAN KE SENDI, OTOT, HEMATOM
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LABORATORIUM* APTT MEMANJANG
* FC VIII MENURUN
PENATALAKSANAAN* UMUM
* SUBSTITUSI FC VIII* KRIOPRESIPITAT
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KOAGULASI INTRAVASKULAR DISEMINATA( K I D )
• Sinonim Konsumsi koagulopati, hiperfibrinolisis, defibrinasi, sindr. Trombohemoragi
• Etiologi KID Fulminan• Bidang Obgin : emboli cairan amniom, abrupsi plasenta, eklaqmsia,
abortus, IUFD• Hematologi : RX transfusi, hemolisis berat, leukemia M3-4• Infeksi : septikemia, viremia, parasitemia• Trauma, luka bakar• Alat prostesis• Penyakit hati akut• Kelainan vaskular
KID derajat rendah• Keganasan, Penyakit autoimun, GVHD,• Penyakit kardiovaskular, Penyakit hati/ginjal kronis,
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PATOFISIOLOGI
XII
Kerusakan Endotel Kolagen Prekalikerin KininogenXIIa
Kompleks Ag-Ab Kalikrein Kinin
XI perm , hipotensi, syok
EndotoksinXia
X Xa Plasminogen PlasminKerusakan jaringan
Aktifitas tromboplastin + VII ProtrombinKerusakan trombosit P.F.1.2 Komplemen
FosfolipidFibrinogen FDP lisis
eri/trombositADP Trombin
Fibrin D. Dimer
Kerusakan eritrosit
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Tanda dan Gejala KlinikA. Plasmin generation (haemorrhage) * Spontaneous bruising * Petechiae * Gastrointestinal bleeding * Respiratory tract bleeding * Persistent bleeding at venepuncture sites * Bleeding at surgical wounds
* Intracranial bleeding
B.Thrombin generation (thrombosis) * Renal failure * Coma * Liver failure * Respiratory failure * Skin necrosis * Gangrene
* Venous thromboembolism
C. Cytokine and kinin generation (shock)* Tachycardia * Hypotension * Oedema
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PATOFISIOLOGI
SYSTEMIC ACTIVATION OF COAGULATION
INTRAVASCULAR DEPOSITION
OF FIBRINDEPLETION OF PLATELETS
BLEEDINGTROMBOSIS
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PEMERIKSAAN LABORATORIUM
FAAL HEMOSTASIS- Trombosit menurun
- Hapusan darah tepi burr cell & fragmentosit- Protrombin Time (PT) memanjang
- APTT memanjang
BUKTI HIPERAKTIFITAS KOAGULASI / FIBRINOLISIS- D-Dimer meningkat
BUKTI KONSUMSI INHIBITOR- Aktifitas antitrombin menurun
- Protein C menurun- Protein S menurun
DISFUNGSI ORGAN- Ureum / kreatinin meningkat
- LDH meningkat- Analisis gas darah
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SKORING DIC Penilaian adanya kelainan dasar / etiologi terkait DIC(jika tak ada penilaian tidak dilanjutkan)
* Hitung trombosit : > 100.000 = 0 50000 – 100.000 = 1 < 50.000 = 2
* D-Dimer : < 500 = 0 500 – 1000 = 2 > 1000 = 3
* Protrombin Time : < 3 detik = 0 4 – 6 detik = 1 > 6 detik = 2
* Fibrinogen : < 100 mg/dl = 1 > 100 mg/dl = 0
Jumlah : ≥ 5 sesuai DIC, < 5 sugestif DIC
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PENATALAKSANAAN* Terapi penyakit dasar* Antikoagulan Heparin / LMWH* Terapi pengganti komponen darah
- FFP : 10 – 15 ml / kgbb- Trombosit- PRC / WRC- Kriopresipitat : bila hipofibrinogenemia
( 10 kantong naik 60 – 100 mg )
* Antitrombin III : Tidak direkemendasikan serentak heparin
* Anti fibrinolitik : tidak direkomendasikan