7.4. polisitemia & dic
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7/21/2019 7.4. Polisitemia & DIC
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FAIZAL DRISSA HASIBUAN
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PENDAHULUAN
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DEFINISI
Polisitemia didefinisikan sebagai
peningkatan jumlah sel darah merah
( eritrosit ) dan beberapa komponendarah.
Definisi lama : peningkatan konsentrasi
hemoglobin diatas nilai normal berdasarkelompok umur atau jenis kelamin
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Nilai Hemoglobin dan Hematokrit normal terendah
Umur dan jenis kelamin Hb Ht
Anak - anak 6 bulan – 6 tahun 11 36
Anak – anak 6 tahun – 14 tahun 12 38
WHO Group Experts on Nutritional Anaemia.
Laki-laki dewasa 13 40
Wanita dewasa tidak hamil 12 38
Wanita dewasa hamil 11 36
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Packed Cell Volume
Laki-laki > 51 %
Wanita > 48 %= Polisitemia
Red Cell Mass > 25 % dari
prediksi berdasar umur dan berat
badan
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KLASIFIKASI POLISITEMIA
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POLISITEMIA VERA
ANAMNESA :
Sering dikeluhkan penderita : nyeri kepala, migrain
yang hebat, wajah yang memerah, rasa gatal
berulan ada tubuh out.
Berhubungan dengan riwayat gangguan vaskular
seperti stroke, iskemik
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KRITERIA DIAGNOSTIK POLISITEMIA VERA
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PENATALAKSANAAN
PRINSIP MENSUPRESI SUMSUM TULANG DAN
PHLEBOTOMI : a o erap a an ur an ag
Hidroksi Urea Trombositosis
Busulfan PLT > 400.000/ uL
PHLEBOTOMI : mempertahankan PCV < 45 % dari
sebelumnya. Tiap 6-10 minggu
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DisseminatedIntravascular
Coagulation
FAIZAL DRISSA HASIBUAN
KOAGULASI INTRAVASKULARDISSEMINATA
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PENDAHULUAN
Sistem Hemostasis
1. Sistem pembuluh darah
. s em rom os
3. Sistem pembekuan darah
4. Sistem fibrinolisis
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1. Sistem pembuluh darah
Fungsinya:
1. Kontraksi pembuluh darah.
2. Aktivasi pembekuan darah dg memproduksi
trombo lastin.
3. Aktivasi trombosit dg memproduksi faktor von
Willebrand.
4. Trombotik : melepaskan aktivator plasminogen.
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2. Sistem trombosit
Fungsinya:
1. Memelihara supaya pembuluh darah tetap utuh
setelah mikro trauma pada endotel.
2. Mengawali penyumbatan pembuluh darah dg.
3. Stabilisasi sumbat trombosit (fibrin), melalui
beberapa tahap:
Adhesi trombosit.
Agregasi trombosit.
Reaksi pelepasan (release).
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3. Sistem pembekuan darah
Pembekuan terjadi o.k interaksi antara pro
koagulan (faktor pembeku), fosfolipid dan
ion. Pro koagulan al:
1. Substrat : fibrinogen (F I).
2. Ko faktor : F III, F V, F VIII, HMWK.3. Enzim : faktor koagulasi yang lain.
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4. Sistem fibrinolisis
1. Proaktivator plasminogen diobah
menjadi aktivator plasminogen.
2. Aktivator plasminogen akan mengobahplasminogen menjadi plasmin.
3. Plasmin menghidrolisis fibrinogen dan
fibrin menjadi fibrin(ogen) degradationproduct (FDP).
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DEFINISI
“ ….an acquired syndrome characterized by the intravascular activation of
coagulation with loss of localization arising from different causes. It can
originate from and cause damage to the microvasculature, which if”, …
Scientific and Standardization Committee of the International Society on Thrombosis
and Haemostasis, Paris July 2001
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Patofisiologi
Kerusakan pembuluh darah akan merangsang proses
koagulasi dan pembentukan fibrin, diikuti oleh
fibrinolisis.
Aktivasi koagulasi berlebihan, mengakibatkanpenggunaan (konsumsi) faktor pembekuan dan
trombosit berlebihan dan akibatnya terjadi :
Pembentukan trombus dimana-mana
Kekurangan faktor pembeku dan
Kekurangan trombosit
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Gejala klinis
Perdarahan difus karena defisiensi faktor
pembekuan dan kekurangan trombosit.
Penyumbatan pembuluh darah dimana- mana, menyebabkan gangguan fungsi
organ vital seperti ginjal dan otak.
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Penyebab
Infeksi : sepsis.
Keganasan:
Ca prostat.
Ca pankreas.
Adeno Ca penghasil mucin.
Lekemia akut:
Acute promielocytic leukemia APL=AML M3)
Komplikasi kehamilan:
Solutio plasenta Operasi
Preeklamsia /eklamsia.
Abruptio plasenta.
HELLP syndrome. Dll.
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Penyebab (2)
Trauma.
Emboli cairan amnion, bekuan darah, udara.
Heat stroke. Gigitan ular.
Penyakit hati.
Kegagalan sirkulasi dan asidosis.
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Laboratorium
Hitung trombosit.
PT.
aPTT. TT.
Kadar fibrinogen.
FDP. D-dimer.
Darah tepi : cari fragmentasi eritrosit.
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Evidence of procoagulant activation
Evidence of fibrinolytic activation
Evidence of inhibitor consumption Evidence of end-organ damage or failure
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Evidence of procoagulation
Elevated prothrombin fragment 1+2
Elevated fibrinopeptide A
Elevated fibrinopeptide B Elevated thrombin-antithrombin complex
Elevated D-dimer
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Evidence of fibrinolytic activation
Elevated D-dimer
Elevated FDP
Elevated plasmin antiplasmin complex
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Evidence for inhibitor consumption
Decreased AT-III
Decreased alpha-2-antiplasmin
Decreased heparin cofactor II Decreased protein C or S
Elevated TAT complex
Elevated PAP complex
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Evidence for end-organ damage
Elevated LDH
Elevated creatinine
ecrease p
Decreased paO2
Needs 2 out of 4 above items for diagnosis
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Terapi
Obati penyakit dasar.
Transfusi dengan Fresh frozen plasma.
Transfusi Trombosit. Pemberian antikoagulan : Heparin.
Tindakan suportif paling penting.
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DIC Score
Platelet count
> 100 = 0; < 100 = 1; < 50 = 2
Elevated fibrin-related marker
no increase = 0; moderate increase = 2;strong increase = 3
Prolonged prothrombin time
< 3 sec = 0; > 3 sec but , 6 sec = 1;
> 6 sec = 2
Fibrinogen level
> 1.0 g/L = 0; < 1.0 g/L = 1
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If > 5 : compatible with overt DIC –
repeat scoring daily
If < 5 : suggestive
(not affirmative) for non-overt DIC –
repeat next 1-2 days