Diatesis Hemoragis (KULIA 4 IPD III).ppt

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