4. Hemostatic Disorder

8/20/2019 4. Hemostatic Disorder

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HEMOSTATIC DISORDER


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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!


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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


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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


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#. -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.


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+. Cliical feat,re' ) == m,co,' mem/rae

/leedi* epi'ta


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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


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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


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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


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. 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


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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!


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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


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/. Diao'i' ) mi


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COAGULATION REGULATION

AND

HYPERCOAGULABLE STATES


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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.


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#. 2i/riolitic '!'tem

%. Acti-atio )

a. Itri'ic acti-atio ) a poorl! ,der'tood

mechai'm of acti-atio

/. E


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+. 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!


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#. 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'


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/. &,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


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/. 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.


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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'


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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


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+. 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


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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


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#. Throm/otic throm/oc!topeic p,rp,ra

(TT&" ad hemol!tic8,remic '!drome

(H@S"

C. Hepari throm/oc!topeia


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4. Hemostatic Disorder

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