diatesis hemoragis (kulia 4 ipd iii).ppt
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HEMOSTASIS - DIATESIS HEMORAGIS
- TROMBOSIS
Vaskular
Trombosit Koagulasi
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
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
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
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
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
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
TROMBOSITOPENI
PRODUKSI KONSUMSI
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
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
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
DIAGNOSIS
ANAMNESIS
PEMERIKSAAN FISIK
LABORATORIUM * DARAH RUTIN
* FAAL HEMOSTATIK* BMP
* PETANDA IMUN
PENATALAKSANAAN
- TRANSFUSI TROMBOSIT - HINDARI TRAUMA / DRUG INDUCED- KORTIKOSTEROID- IMUNOGLOBULIN
BILA REFRAKTER - IMUNOSUPRESIF
SIKLOFOSPAMID, AZATIOPRIN, VINKRISTIN- SPLENEKTOMI
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
LABORATORIUM* APTT MEMANJANG
* FC VIII MENURUN
PENATALAKSANAAN* UMUM
* SUBSTITUSI FC VIII* KRIOPRESIPITAT
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,
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
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
PATOFISIOLOGI
SYSTEMIC ACTIVATION OF COAGULATION
INTRAVASCULAR DEPOSITION
OF FIBRINDEPLETION OF PLATELETS
BLEEDINGTROMBOSIS
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
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
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