Lbm 3 Hemostasis Sgd 17 .Dox

7/21/2019 Lbm 3 Hemostasis Sgd 17 .Dox

1/23

LBM 3 HEMOSTASIS

STEP 1

- Ptechiae : small 1 until 2 mm small red or purple spot inthe body becausd of a minor hemorhage

- Blood clotting disorder : the blood cant stop normaly

- Trauma : feel scared that never feel again

- Fever : reaction the body because infection

STEP 2

1. What the factors that aect blood clotti! "

2. What the #echais# of he#ostasis "

3. Wh$ the blood cat sto% or#al$ "

&. Whe the %rocess of he#ostasis occ'red "

(. What abor#alities i he#ostasis "

). What are the ca'se of he#ostasis "

*. What the #or%holo!$ ad +siolo!$ of %latelet "

,. What -id of he#o+lia ad e%lai it "

/. Ho0 relatio bet0ee fro# he#ostatis ad bloodclotti! "

1. What are the resfactors ad 0hat is

-oa!'latio st'dies "

11. What are the basic dia!ostic dasar dia!osis

%e#be-'a darah berdasar-a a%a


2/23

12. Maa4e#e 5 %ea!aa dari he#ostasis

13. Pe#eri-sa $a 6 tro#bosit$a ba!ai#aa 6

-oa!'lasi$a ba!ai#aa 7as-'larisasi$a se%erti

a%a

STEP 3


- Koagulation factors

- Christmast factors : protease serin from hemostatis

- PT! " Plasma Tromboplastin !ntecendent # : protease serin

from hemostatis

- !nti $emo%li& factors : co factor from hemotasis

- Fletcher factors: the activation contact

- Fit'gerald factor :The activation contact

- (nfection : decrease the production of %broblast

- Forage body ) benda asing :

- *2 : needed for the sintesis of colagen

- +mo&e : caused a lac& of o,igen in the blood

- Preasure : ound here sutured to tight ill cause

necrotic

- Blood supply : ade.uate blood supply nedeed to supply

o,igen to nutrient

- Temperature

- Protein and carbohidrat


3/23

- /utrient status

- 0e%siensi of vitamin


asoconstriction

(mmediate vasoconstriction of the bloof vessels and

inured and re3e, constriction of the small arteries

and arterioles in the vicinity caused the initial

slodon of blood

The reaction of platelet and formation of the primary

+tabili'ation of the plateler plug by %brin


Because the hemostasis pocces cant or& normaly

Because the funtion of tromocyte decrease

(t caused by a de4ect in one of the genes that determind

ho the body ma&es blood clotting factors ((( or (5

(f one of them have a problem caused blood clotting

normaly for e,ample hemo%lia ! and B


6hen our body getting inure and the blood out from our

body and the factors of blood clotting or&

6hen blood vessels ruptured

(. What abor#alities i he#ostasis "


4/23

Tromositosis : hen the platelet is more than normal 7

189999 ;89999

$emo%lia : is genetics deseases that inheretage

based on , lin&ed resesif

Thrombositopenia : hen platelet count of less than

199999)mmorphology : no nucleus 7 disc shape 7 si'e 2-


6/23

STEP *


Essentials of Human DiseaseBy Leonard Crowley



7/23

a#Primary

>echanism of vasoconstrictionof blood vesselsin the small ound

b# +econdary

>echanism in volving coagulation factorsin plasmaandplateletsith the

ultimate goalnets%brinformation7 occurs ina largeound

c#Tertiary

Control mechanismthat preventse,cessivehemostasisnot

through%brinolyticsystem

Sumber: http://digilib.unimus.ac.id/files/disk1/125/jtptunimus-gdl-

adityadwid-621-2-babii.pdf


8/23


8'e to a blood clotti! disorder.I a or#al %erso 0he i4'red6 %latelets 0ill stic-

to each other ad for# a!!re!ates that co'ld sto%

the blood co#i! o't. B't there are also %eo%le

0ho !et h'rt a little6 the blood -e%t %o'ri! or

di9c'lt berheti.bila e%erieci! these coditios6


9/23

it #eas there is a %roble# 0ith the %rotei i red

blood cells is called the 7o Willebrad :W;.:o Willebrad disease :W8 is oe of the

bleedi! disorder !ee dit'r'a- fro# his%arets. :o Willebrad factor is i#%ortat beca'se

it cotrols bleedi! ad blood clotti! %rocess.I#%ortat for cotrolli! bleedi! ad blood

clotti! %rocess.

If $o' do ot ha7e these factors6 the %latelets that

there 0ill ol$ ho7er or #a$ ot be able to stic- toad 0ill ot for# a!!re!ates that coti'o's

bleedi! occ'rs.Kapita Selekta Hematologi A.V. Hofbrand Edisi 4


1asoconstriction due to local neural response7 and release ofendothelin from the endothelium

2Primary hemostatic plug: due to platelet adhesion7 activation7degranulation"!0P7 T5!2# and recruitment of other platelets


10/23

mutations in the F@ gene cause hemophilia !7 the most common form of

this bleeding disorder >ore than 17


11/23

ith early diagn#sis% pe#ple with "#n illebrand disease can lead n#rmal% acti"e

li"es. ,e#ple with mild cases may n#t re0uire treatment% but sh#uld a"#id taking drugs

that c#uld aggra"ate bleeding% such as aspirin and ibupr#fen% with#ut first c#nsulting

with a d#ct#r. #re seri#us cases may be treated with drugs that increase the le"el #f

"#n illebrand fact#r in the bl##d #r with infusi#ns #f bl##d fact#r c#ncentrates. !t is

imp#rtant f#r pe#ple with "#n illebrand disease t# c#nsult with their d#ct#rs bef#re

ha"ing surgery% ha"ing dental w#rk% #r gi"ing birth% s# that pr#per precauti#ns can be

taken t# pre"ent e$cessi"e bleeding. #u may be referred t# a hemat#l#gist% a d#ct#r

wh# speciali7es in the treatment #f bl##d dis#rders.

+umber : http://www.hemat#l#gy.#rg/,atients/8l##d-9is#rders/8l##d-l#ts/52.asp$%

http://faculty.ucc.edu/bi#l#gy-p#tter/hem#stasis.htm ; biosbccnet

Tro#bosito%eia %e'r'a 4'#lah tro#bosit

Etiologi

a ?angguan produ&si trombosit oleh mega&ariosit- 0epresi mega&ariosit &arena obat7 bahan &imia7 virus- Bagian dari &egagalan sum-sum tulang :

o !nemia aplasti&

o Aeu&imian a&ut

o +indrom mielodisplasiti&b Penghancuran trombosit di darah tepi

- (diopathic thrombocytopenic purpura "(TP#- 0isseminated intravascular coagulation "0(C#- 0rug induced immune trhrombocytopenia

c 0istribusi tida& norma+indrom hipersplenisme : terado pooling trombosit dalam liend !&ibat pengenceran!&ibat transfusi masif

Tro#bo%ati -elaia f'!si tro#bosit1 Trombopati herediter

a Platelet pool storage disease = gangguan pelepasan !0P

gangguan agregasi trombositb Tromboasthenia ?lan'man= gangguan reseptor ?P ((b-(((a pada

permu&aan trombosit agregasi gagal = autosomal resesifc +indrom bernard-soulier= gangguan reseptor ?P (b tida& teradi

adhesi dg v6Fd Penya&it von 6illebrand= tida& terbentu& v6F tida& teradi adhesi

platelet2 Bentu& didapat

a !&ibat terapi aspirin= gangguan sintesis thrombo&sin !2

mencegah agregasib $iperglobulinemia
http://www.hematology.org/Patients/Blood-Disorders/Blood-Clots/5233.aspxhttp://www.hematology.org/Patients/Blood-Disorders/Blood-Clots/5233.aspxhttp://www.biosbcc.net/http://www.biosbcc.net/http://www.hematology.org/Patients/Blood-Disorders/Blood-Clots/5233.aspx


12/23

c Kelainan mielopoliferatifd ?agal ginale Penya&it hati menahun

:o Willebrad=s 8isease

Timbul &arena sintesis v6F menurun 7 menga&ibat&an :

- ?angguan adhesi trombosit- >enurunnya a&tivitas F((( C dalam plasma

Klasi%&asi :

1 Tipe ( : penurunan sintesis v6F2 Tipe (( a : gangguan sintesis multimer v6F besar dan sedangTipe (( b : pembentu&an multimer v6F besar dan sedang sehingga cepat

di&eluar&an dari darah< Tipe ((( : sintesis v6F sama se&ali tida& ada

Manifestasi Klini&

- Perdarahan sedang7- epista&sis sea& &ecil7- menorrhagi- perdarahan dari lu&a- postoperasi- Perdarahan besar- $ematom

He#o+li A da B

$emo%li ! dan B merupa&an gangguan fungsi &oagulasi herediter

Patogenesis :

1 $emo%li ! disebab&an def F((( clotting activity "F(((C# dapat &arena

sintesis menurun atau pembentu&an abnormal2 $emo%li B7 &arena def F(5

8e+siesi :it


13/23

< Fungsi vit K dihambat oleh anti &oagulan

$ematologi Klini& E

&.8I>8isse#iated Itra7asc'lar >oa!'latio

Adalah s'at' sidro#a -lii- $a! disebab-a oleh

de%osisi +bri siste#i- da %ada saat $a! sa#a

ter4adi -eceder'!a %erdaraha da tro#bosis.

He#atolo!i ade Ba&ta

). What are the ca'se of he#ostasis "

!bnormal bleeding can occur due to a variety of vascular system disorders

either hereditary or ac.uired This disorder is most seringdiumpai cause

bleeding in the clinic sually a mild s&in bleeding and lasted &uranglebih

;@ hours

The cause of this disorder could be due to:

-structure of abnormal blood vessels

-the process of in3ammation or immune reaction

- !bnormal tissue perivas&uler

He#atolo!i ade Ba&ta

*. What the #or%holo!$ ad +siolo!$ of %latelet "


14/23


15/23


16/23

his person has low levels of or is missing completely factor ** (Also called FI

or factor I deficiency) Hemophilia is *0 times more rare than type A! Factor

I deficiency is different ,eca"se it can show "p in ,oth males and females!


17/23

11. What are the basic dia!ostic dasar dia!osis

%e#be-'a darah berdasar-a a%a


18/23


19/23

12. Maa4e#e 5 %ea!aa dari he#ostasis

a.8e+siesi -o#%le-s fa-tor :III ata' ;a-tor 7o 0illebrad6 d'a dari

!a!!'a herediter %erdaraha $a! seri! dite#'-a6 disebab-a

oleh -elaia -'alitatif ata' -'atitatif $a! #e!eai fa-tor

:III5:0f.

8e+siesi fa-tor :III ii #e$ebab-a he#o+li A.

He#o+li A adalah %e$a-it herediter terseri! $a! #e$ebab-a

%edaraha seri's.

Tera%i$a de!a if's fa-tor :III.


20/23

Tabel Klasifikasi dan Gejala Hemofilia

Klasifikasi Gejala Kadar FVIIIHemofilia Aberat

Perdarahan spontan tanpa adapenyebab berupa trauma atautindakan operatif. Perdarahanspontan dapat terjadi pada saatmelakukan aktivitas sehari-hari.Pada umumnya ditemukan pada usiadini bahkan kurang dari satu tahun.

< !" unit # mlplasma

Hemofilia Asedang

Perdarahan yang sukar # tidak dapatberhenti pada trauma atau tindakan

operatif. Pada umumnya terdeteksipada saat ekstraksi gigi atau operasiminor seperti sirkumsisi.

!" $ !% unit # ml

plasma

Hemofilia Aringan

Perdarahan yang sukar # tidak dapatberhenti pada trauma atau tindakanoperatif yang lebih berat.

!%" -!&' unit # mlplasma

b.de+siesi fa-tor I@ He#o+lia B6 %e$a-it christ#as

s'at' %e$a-it $a! secara -liis tida- da%at dibeda-a de!a

he#o+lia A.

Pe$a-it ii dia#a-a %e$a-it christ#as -area berdasar-a

a#a %asie $a! %erta#a #e!ida% %e$a-it ii.

Tera%i$a de!a if's fa-tor I@ $a! saat ii s'dah tersedia secara

l'as.


21/23

B'-' A4ar Patolo!i Edisi * 6?obbis lotti! ti#e5#asa %e4edala

&.4'#lah tro#bosit

(.%roto#bi ti#e 5 %las#a %roto#bi ti#e

).acti7ated %artial tro#bo%lasti ti#e

*.tro#bi ti#e

a.Hitung darah dan pemeriksaan sediaan hapus darah

Memastikan kemungkinan adanya trombositopenia, tindakan ini mungkin dapat

menemukan penyebabnya, missal leukemia akut

b.Uji skrining pembekuan darahMemungkinkan penilaian terhadap system "ekstrinsik"

dan "intrinsik"pembekuan darah juga perubahan sentral fibrinogen menjadi fibrinc.

c.Masa perdarahan

Pemeriksaan yang berguna untuk fungsi trombosit yang abnormal, termasukdiagnosis

defisiensi VWF. Masa perdarahan juga memanjang padatrombositopenia tetapi normal

pada perdarahan abnormal yang disebabkanoleh sebab vasculard.

d.Uji fungsi trombosit


22/23

Pemeriksaan yang paling berguna adalah agregometri trombosit yangmengukur

penurunan serapan cahaya dalam plasma kaya trombosit sejalan dengan agregasi

trombosite.

e.Uji terhadap fibrinolisis

Meningkatnya kadar activator plasminogen yang bersirkulasi dapat dideteksi dengan

dengan adanya pemendekan masa lisis bekuan euglobulin.

Sejumlah metode imunologik untuk mendeteksi produk pemecahanfibrinogen atau fibrin

dalam serum . pada pasien yang mengalamipeningkatan fibrinolisis dapat

dideteksi kadar plasminogen dalam darahyang rendah

HEMATOLOGI KAPITA SELEKTA

13. Pe#eri-sa $a 6 tro#bosit$a ba!ai#aa 6

-oa!'lasi$a ba!ai#aa 7as-'larisasi$a

"diopathic #hromoc$topenic %urpura ("#%)

IT# adalah elainan ai$at trom$ositopenia yan! tida dietahui penye$a$nya %idiopati)& tetapi ternyata

dietahui $ahwa se$a!ian $esar elainan ini dise$a$an oleh proses imun& arena itu dise$ut 'u!aautoimmune

thrombocytopenic purpura.

#ada IT# 'umlah trom$osit menurun dise$a$an oleh trom$osit diiat oleh anti$odi& terutama I!(. nti$odi

terutama ditu'uan untu reseptor (# II$*IIIa pada trom$osit. Trom$osit yan! diselimuti anti$odi emudian

difa!ositir oleh marofa! dalam +, terutama lien& ai$atnya ter'adi trom$ositopenia.

(am$aran lini IT#& yaitu ) onset pelan den!an perdarahan melalui ulit atau muosa $erupa petei&

eimosis& easy bruising& menorrha!ia& epistasis atau perdarahan !usi/ 0) perdarahan # 'aran!& tetapi fatal/

dan 1) splenome!ali& ter'adi pada 23 asus.

#ada IT# elainan la$oratorium yan! ter'adi4 ) darah tepi4 trom$osit palin! serin! antara 2.222-52.222*mm1/ 0)

sumsum tulan!4 me!aariosit menin!at& multinulear& disertai lo$ulasi/ dan 1) imunolo!i4 adanya antiplatelet I!(

pada permuaan trom$osit atau dalam serum. 6an! le$ih spesifi adalah anti$odi terhadap !p II$*IIIa atau !p I$.

7ia!nosis IT# dite!aan $ila di'umpai4 ) !am$aran lini $erupa perdarahan ulit atau muosa/ 0)

trom$ositopenia/ 1) sumsum tulan!4 me!aariosit normal atau menin!at/ 8) anti$odi antiplatelet %I!() positif&

tetapi tida harus demiian/ dan 5) tida ada penye$a$ trom$ositopenia seunder %Bata& 0229).

1. Hemophilia A (classic hemophilia) - leads to the production of a defective Factor VIII. This isthe most common form of hemophilia and is due to a X chromosome-lined recessive !ene.It is most common in males.

2. Hemophilia B (Christmas disease)- leads to the production of a defective Factor IX."emophilia B is due to a defective !ene lined to the X chromosome and is most commonlyfound in males.

3. Von Willerand!s Disease- the result #f a lack #f effecti"e "#n illebrands act#r. !t is

due t# an aut#s#mal d#minant gene and #ccurs e0ually in males and females.


23/23

on 6illebrand disease is an inherited condition that results hen the blood

lac&s von 6illebrand factor7 a protein that helps the blood to clot and also

carries another clotting protein7 factor ((( (t is usually milder than hemophilia

and can a4ect both males and females on 6illebrand disease is classi%ed

into three di4erent types "Types 17 27 and

Documents

Lbm 3 Hemostasis Sgd 17 .Dox