Download - Koagulasi Intravaskuler Disseminata.ppt
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Kelainan pembekuan darah
Dr. Isbandiyah, SpPD
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Hemostasis Hemostasis (proses penghentian perdarahan)
Yaitu usaha tubuh agar tdk kehilangan darah terlalu
banyak bila terjadi luka pada pembuluh darah dan darah
tetap air dan mengalir seara lanar
!eberapa ara " Spasme pembuluh darah
Pembentukan sumbat trombosit
Pembentukan bekuan darah Pertumbuhan jaringan ikat ke dalam bekuan darah
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hemostasis !eberapa komponen hemostasis
Pembuluh darah
#rombosit
$aktor pembekuan
$ibrinolisis
% maam hemostasis" Hemostasis primer
Hemostasis sekunder (koagulasi)
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Pembekuan darah &kti'asi pembekuan darah dapat melalui
jalur"
alur intrinsik
alur ekstrinsik
alur bersama
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Kaskade Koagulasi
alur intrinsik alur ekstrinsik
HMWK
Prekalikrein
Faktor XII
XI XIa
IX IXa
X Xa
II IIa
Fibrinogen Fibrin FDPs
Kerusakan endotel / jaringan
Tissue factor
VII VIIa
VIII
V
Plasminogen Actie !las"in
Tissue !las"inogen
actiator
#ross$linked fibrin
XIII
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De*isiensi *aktor+*aktor pembekuan Hemo*ilia & (de*. *aktor III)
Hemo*ilia ! (de*. *aktor I-) on illebrands (de*. *aktor III
dan gangg. $aal trombosit)
De*isiensi it. K Penyakit hati
DI/
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Hemo*ilia & &dalah kelainan herediter -+linked resesi*
dengan karakteristik de*isiensi *aktor III
0tiologi" mutasi gen pada kromosom -" -1%2
3ejala klinis" mudah terjadi perdarahan
Diagnosis" kadar *aktor III menurunPemanjangan P##, /#
#erapi" penambahan *aktor III
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Hemo*ilia ! Kelainan ba4aan dmn terjadi de*isiensi
*aktor I-
35 klinis" sama dengan hemo*ilia &
D-" pemanjangan P##
Kadar *aktor I- menurun #erapi" diberikan *aktor I-
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Koagulasi Intra'askular Disseminata
Pengertian " &kti'itas sistem koagulasi dan *ibrinolisis seara berlebihan dan
terjadi pada 4aktu yang bersamaan
Diagnosis
Klinis 3ejala umum " demam, hipotensi, asidosis, hipoksia dll
#rombosis 6 gagal organ ( paru, hati, ginjal )
3ejala kausa primer
7bstetri " emboli airan amnion, I8$D, septi abortion
Hematologi 6 reaksi trans*usi, hemolisis berat, leukemia
In*eksi " sepsis,HI, hepatitis, dengue, malaria
#rauma, penyakit hati akut, luka bakar
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Pato*isiologi KID
Deposit *ibrin pada mikrosirkulasi
Iskemia jaringan Kerusakan dan hemolisiseritrosit
$ibrinolisis sekunder
Perdarahan menyeluruh
Konsumsi trombosit dan*aktor koagulasi
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Pemeriksaan penunjang
Darah tepi " trombositopeni atau normal Pemeriksaan hemostasis 6 P##+aP##, *ibrinogen, d+dimer
#erapi
Suporti* 9emperbaiki dan menstabilkan hemodinamik
9embebaskan jalan na*as
Keseimbangan asam basa
Keseimbangan elektrolit
9engobati penyakit primer
9enghambat proses patologis " antikoagulan (heparin),
trans*usi komponen darah sesuai indikasi
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Komplikasi
3agal organ
Syok:hipoper*usi #rombosis 'ena dalam
Prognosa
Dubia at malam
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