4. Hemostatic Disorder
-
Upload
imam-muhamad-rissandy -
Category
Documents
-
view
222 -
download
0
Transcript of 4. Hemostatic Disorder
-
8/20/2019 4. Hemostatic Disorder
1/26
HEMOSTATIC DISORDER
-
8/20/2019 4. Hemostatic Disorder
2/26
I. CONGENITAL HEMORRHAGIC DISORDER
A. Hemophilia A (factor VIII deficiec!" adHemophilia # (factor I$ deficiec!"
%. &athoph!'iolo! )
$ chromo'ome* ee defect.+. Cliical feat,re' )
mild* moderate ad 'e-ere di'ea'e
. Diao'i' )
a. 'creei te't'
/. 'pecific factor a''a!
-
8/20/2019 4. Hemostatic Disorder
3/26
0. Therap! ) to rai'e the deficiet factor
a. &harmacoloic therap! )
%. Hemophilia A ) De'mopre''i (dDAV&"
+. Hemophilia # ) o effecti-e dr,'
/. Replacemet therap! )
%. #e1are of ad-er'e effect'
+. The choice of /lood prod,ct i' critical
-
8/20/2019 4. Hemostatic Disorder
4/26
c. Treatmet optio' )
%. 2re'h fro3e pla'ma (22&"
+. Cr!oprecipitate
. 2actor cocetrate'
4. Complicatio ) Arthropath!* Ihi/itor'*
Li-er di'ea'e* HIV ifectio.
5. Iterdi'cipliar! care → hemophilia ceter medical care* p'!cho'ocial care ad
eetic co,'eli
-
8/20/2019 4. Hemostatic Disorder
5/26
#. -o 6ille/rad7' di'ea'e
%. &h!'iolo! of -62 )
8 HM6 l!coprotei* +49 :D
8 I pla'ma ad i platelet'
8 a' a carrier protei for coa. 2 VIII
8 importat for primar! hemo'ta'i'
8 mediate' adhe'io of platelet' to the '.edot8 -62 ; 2 VIII comple< ) 2 VIII ) C* 2 VIII ) A*
2 VIII ) Ca* 2 VIII ) RCof.
-
8/20/2019 4. Hemostatic Disorder
6/26
+. Cliical feat,re' ) == m,co,' mem/rae
/leedi* epi'ta
-
8/20/2019 4. Hemostatic Disorder
7/26
Ta/le +. La/orator! 2idi' i Hemophilia A
ad Se-ere -62 Di'ea'e
La/otator! te't 2idi
Hemophilia A -62 di'ea'e
#leedi time
VIII ) C
VIII ) A
VIII ) RCof
Normal
Decrea'ed
Normal
Normal
&roloed
Decrea'ed
Decrea'ed
Decrea'ed
-
8/20/2019 4. Hemostatic Disorder
8/26
0. Therap!
a. dDVA&
/. Cr!oprecipitate
c. 2 VIII cocetrate'
C. Other iherited factor deficiecie'
%. 2 $II* pre:alli:rei ad HM6>
+. 2 I* II* V* VII* $* $I ad $III
-
8/20/2019 4. Hemostatic Disorder
9/26
II. AC?@IRED HEMORRHAGIC DISORDER
A. Vitami > deficiec!%. Etiolo!
a. dietar! deficiec!
/. mala/'orptioc. ati/iotic therap!
d. hemorrhaic di'ea'e of the e1 /or
+. Cliical feat,re' ) 'e-ere /r,i'i or
e
-
8/20/2019 4. Hemostatic Disorder
10/26
. Diao'i'
8 'e-ere ) proloed &T ad &TT
8 earl! or milder def. ) proloed &T
0. Therap!
8 &areteral -it. >8 22&
#. Li-er di'ea'e
%. Etiolo!a. decrea'ed '!the'i' of coa. factor'
/. Vit. > def
-
8/20/2019 4. Hemostatic Disorder
11/26
c. 2,ctioall! a/ormal fi/rioe'
d. Di''emiated Itra-a'c. Coa,latio(DIC" ad co',mptio of coa. factor'
+. Cliical feat,re'
8 -ar! 1ith the co,r'e of the patiet7' li-er
di'ea'e
8 == GIT /leedi. Diao'i'
8 La/. fidi' → -arie' 1idel!
-
8/20/2019 4. Hemostatic Disorder
12/26
0. Therap!
8 trial of pareteral -it > therap!
8 Replacemet therap! → 22&
C. Clotti factor ihi/itor' → a,toati/odie'
%. Ihi/itor' i hemophilia+. Ihi/itor' i patiet' 1itho,t pree
-
8/20/2019 4. Hemostatic Disorder
13/26
/. Diao'i' ) mi
-
8/20/2019 4. Hemostatic Disorder
14/26
COAGULATION REGULATION
AND
HYPERCOAGULABLE STATES
-
8/20/2019 4. Hemostatic Disorder
15/26
I. CONTROL MECHANISMS IN COAG@LATION
A. Nat,rall! occ,ri a.coa,lat'
%. Atithrom/i III (AT III" → act' a' a 'erie
protea'e ihi/itor +. &rotei C* &rotei S → Vit > ; depedet
. Other pla'ma protea'e ihi/itor ) hepari
cofactor II* 28 macrolo/,li.
-
8/20/2019 4. Hemostatic Disorder
16/26
#. 2i/riolitic '!'tem
%. Acti-atio )
a. Itri'ic acti-atio ) a poorl! ,der'tood
mechai'm of acti-atio
/. E
-
8/20/2019 4. Hemostatic Disorder
17/26
+. Site' of actioa. 2i/rioe
/. 2i/ri
II. THROM#OTIC DISORDER
A. Coeital throm/otic di'order
%. AT III deficiec!
+. &rotei C deficiec!
. &rotei S deficiec!
-
8/20/2019 4. Hemostatic Disorder
18/26
#. A?@IRED THROM#OTIC DISORDER
→ THROM#OEM#OLIC DISEASE
%. Ri': factor' )
a. a/ormalitie' of /lood flo1
/. a/ormalitie' of the -a'c,lat,rec. a/ormalitie' of the coa,latio '!'tem
+. Cliical maife'tatio' )
a. Veo,' throm/o'i' 8 ',perficial throm/o'i'
8 deep -ei throm/o'i'
-
8/20/2019 4. Hemostatic Disorder
19/26
/. &,lmoar! em/oli'm
c. Arterial throm/o'i'
d. Other 0. Therap!
a. Aet'→ treatmet ad pre-etio
%. Aticoa,lat' )8 Hepari
8 6arfari
+. Throm/ol!tic aet' )8 Strepto:ia'e
8 @ro:ia'e
8 t8&A
-
8/20/2019 4. Hemostatic Disorder
20/26
/. Treatmet approache'
%. Deep -ei throm/o'i' ) == hepari*
throm/ol!tic aet
+. &,lmoar! em/ol,' ) hepari* throm/ol!tic
aet
. M!ocardial ifarctio ) throm/ol!tic aet
0. &eripheral arter! ad catheter throm/o'i' )
throm/ol!tic aet.
-
8/20/2019 4. Hemostatic Disorder
21/26
III. THROM#OHEMORRHAGIC DISORDER
A. Di''emiated itra-a'c,lar coa,latio(DIC"
%. &athoph!'iolo! ('ee ta/le "
a. Iitiatio ) patholoic acti-atio of
the coa. ca'cade
/. Throm/o'i'
c. Co',mptio d. 2i/riol!'i'
e. Hemol!'i'
-
8/20/2019 4. Hemostatic Disorder
22/26
Infectious condition
Gram negative septicemia
Other endotoxin-related conditionObstetric conditions
Abruptio placentae
Amniotic fluid embolism
Retained dead fetusVascular conditions
Aneurysm
Giant cavernous hemangioma
Hematologic conditions
assive hemolysis
!romyelocytic leu"emia
#na"e venom
$rauma
Ta/le . Coditio' that commol! precipitate DIC
-
8/20/2019 4. Hemostatic Disorder
23/26
+. Cliical feat,re' → -arie' 1idel!
a. diff,'e /leedi from m,ltiple 'ite'
/. throm/otic le''io'
. Diao'i' → la/. fidi' -ar! 1ith time Bcirc,m'tace'
Se-ere DIC )
a. Throm/oc!topeia
/. &roloed &T* &TT* TT
c. Decrea'ed fi/rioe
d. 2S&'
e. Microaiopathic hemol!tic aemia
-
8/20/2019 4. Hemostatic Disorder
24/26
0. Therap! )
a. Lo1 rade DIC→ treatmet ma! ot
/e ece''ar!
/. Cliicall! 'iificat /leedi →
replacemet of depleted coa,latio
factor' ad cell'c. Throm/o'i' ) hepari
-
8/20/2019 4. Hemostatic Disorder
25/26
#. Throm/otic throm/oc!topeic p,rp,ra
(TT&" ad hemol!tic8,remic '!drome
(H@S"
C. Hepari throm/oc!topeia
-
8/20/2019 4. Hemostatic Disorder
26/26
THANK YOU