modul 2 b7
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SCENARIOSCENARIO
A girl aged 5 years old, wasA girl aged 5 years old, was
brought to the hospital becausebrought to the hospital because
she got red spots on her arm,she got red spots on her arm,
lower extremities and abdomen,lower extremities and abdomen,
blood coming out rom her anusblood coming out rom her anus
with no e!er" # days ago, she hadwith no e!er" # days ago, she had
$ust reco!ered rom cough with$ust reco!ered rom cough withrhinorrhea"rhinorrhea"
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%E&'OR(S%E&'OR(S
girl aged 5 years oldgirl aged 5 years old red spots on her arm, lower extremitiesred spots on her arm, lower extremities
and abdomenand abdomen
blood coming out from her anusblood coming out from her anus no feverno fever
Previous cough and rhinorrhea, 6 daysPrevious cough and rhinorrhea, 6 days
ago.ago.
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)ER*S C+ARIICA)ION)ER*S C+ARIICA)ION
Hemorrhage:the escape blood from the vessel.Hemorrhage:the escape blood from the vessel.
Petechia : a red spot due to escape of a small of blood.Petechia : a red spot due to escape of a small of blood.
Purpura : a small hemorrage in the sin,mucousPurpura : a small hemorrage in the sin,mucous
membrane, ormembrane, or serous surface. serous surface.
!cchymoses:a small hemorrhagic spot in the sin or a!cchymoses:a small hemorrhagic spot in the sin or amucousmucous membrane, larger than a petechia,membrane, larger than a petechia,
forming aforming a nonelevated, rounded, or irregularnonelevated, rounded, or irregular
blue or purplishblue or purplish patch. patch.
"elena"elena : the passage of dar stools stained with: the passage of dar stools stained with
altered blood byaltered blood by the intestinal #uices. the intestinal #uices.Hematoche$ia : the passage of red bloody stoolsHematoche$ia : the passage of red bloody stools
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-.ES)IONS-.ES)IONS
How is the mechanism ofHow is the mechanism of
hemostasis%hemostasis%
How hemmorhage or bleeding canHow hemmorhage or bleeding canoccur%occur%
&hat is the correlation of previous&hat is the correlation of previous
disease with the symptom%disease with the symptom% 'i(erential diagnosis and their'i(erential diagnosis and their
description.description.
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HEMOSTASIS
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BLOOD COAGULATION
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I/RINO+&SIS 0I/RINO+&SIS 0
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FIBRINOLISIS 2
XIIa
INTRINSIK
KALIKREIN
PLASMINOGEN
EKSTRINSIK EKSOGEN
AKTIVATOR
PLASMINOGEN
UROKINASE
TERIKAT
PLASMIN
TERIKAT FIBRIN
FDP
PLASMINOGEN
BEBAS
BEBAS
PLASMINFIBRINNOGEN
F V
F VIII
ANTI PLASMIN
t-PA
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1E*ORR1A2E31E*ORR1A2E3
)ascular abnormality)ascular abnormality
*hrombocyte abnormality*hrombocyte abnormality
+oagulation abnormality+oagulation abnormality 'eciency vitamin -'eciency vitamin -
*rauma*rauma
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(IEREN)IA+(IEREN)IA+
(IA2NOSIS(IA2NOSIS 'isseminated ntravasucular'isseminated ntravasucular+oagulation+oagulation
diophatic *hrombocytopenicdiophatic *hrombocytopenic
PurpuraPurpura
Henoch /choenlein /yndromeHenoch /choenlein /yndrome
von &illebrand 'iseasevon &illebrand 'isease Hemophilia 0Hemophilia 0
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)A/+E)A/+E
'+'+ *P*P H!12+HH!12+H/+H2!13/+H2!13!1!1/41'2"!/41'2"!
)21!&33)21!&33!01'!01'/41'2"!/41'2"!
H!"2PH30H!"2PH3000
P!*!+H0P!*!+H0 77 77 77 77
8P9P908P9P90
;;
H!"0*2+H!
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(ISSE*INA)E((ISSE*INA)E(IN)RA4ASC.+ARIN)RA4ASC.+AR
COA2.+A)IONCOA2.+A)ION
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DefnitionDefnition
Disseminated intravascular coagulation (DIC) is a
complex systemic thrombohemorrhagic disorder
involving the generation of intravascular fibrin
and the consumption of procoagulants and
platelets.
DIC is a syndrome arising as a complication ofmany different serious and life-threatening
illnesses.
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CON)CON)
/ynonim: +onsumption coagulopathy ,/ynonim: +onsumption coagulopathy ,
Hiperbrinolytic activation,Hiperbrinolytic activation,debrination anddebrination and
thrombohemorragic disorderthrombohemorragic disorder
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ET IOLOGYET IOLOGY 2bstretical complication:2bstretical complication:
; abruptio placentae; abruptio placentae
; amniotic Auid embolism; amniotic Auid embolism
; dead fetus syndrome; dead fetus syndrome
;; Septic abortionSeptic abortion *rauma : ; burns*rauma : ; burns nfection: ; bacteria /epsisnfection: ; bacteria /epsis
; viral viremia; viral viremia mmunologic B lood transfusion mistaemmunologic B lood transfusion mistae "alignancy, example : 3euemia"alignancy, example : 3euemia
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PATOMECANISPATOMECANIS
MMPrecipitating "echanismPrecipitating "echanism
*issue damage !ndothelial*issue damage !ndothelial
damagedamage
*issue tromboplastin /timulate*issue tromboplastin /timulate
intrinsicintrinsic
8> pathway of8> pathway of
coagulationcoagulation
!xtrinsic pathway of 8>. C from blood!xtrinsic pathway of 8>. C from blood
plasmaplasma
+oagulation protein+oagulation protein
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'isseminated'isseminated
+oagulation process+oagulation process
2cclusion ntravascular trombin; brin DD2cclusion ntravascular trombin; brin DD+onsumption+onsumption
blood vessel ; +oagulationblood vessel ; +oagulationfactorfactor
; platelet.; platelet.
*issue ischemic >ibrinolysis DD8Homeostasis*issue ischemic >ibrinolysis DD8Homeostasis
=organs 777=organs 777
educe:educe: >'P ; +oagulation factor>'P ; +oagulation factor
; platelet; platelet
1ecrosis1ecrosis
coagulation inhibitedcoagulation inhibited
leedingleeding
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CLINICALCLINICAL
SYMPTOMSSYMPTOMS'+ BD *rombosis and bleeding can be happen together'+ BD *rombosis and bleeding can be happen togetherE. Hemorrhagic diatesis : leeding preference.E. Hemorrhagic diatesis : leeding preference.
; 'ermatology : Petechiae, ecchymosis of sin or; 'ermatology : Petechiae, ecchymosis of sin ormucous membranemucous membrane
nger ganggrenous.nger ganggrenous. ; ?astro : Hematemesis, hematoche$ia, and visceral; ?astro : Hematemesis, hematoche$ia, and visceral
hemorrhagehemorrhage
; ?enitourinary : Hematuria, metrorrhagia.; ?enitourinary : Hematuria, metrorrhagia.
; ?inggival bleeding.; ?inggival bleeding.
F. 'ispneuF. 'ispneu
G.G. tachycardia, tachypnea, and hypotensiontachycardia, tachypnea, and hypotension5.5. In severe cases, patients may develop feverIn severe cases, patients may develop fever
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LABORATORY FINDINGLABORATORY FINDING
lood sho!s an abnormalities coagulation because of fibrinogenlood sho!s an abnormalities coagulation because of fibrinogen
deficiency.deficiency.
"emostatic #xamination $"emostatic #xamination $
%. &latelet count decreased%. &latelet count decreased'. Decreased fibrinogen level'. Decreased fibrinogen level
. &rolonged prothrombin time, activated partial. &rolonged prothrombin time, activated partial
thromboplastin time, thrombin timethromboplastin time, thrombin time
. Increased levels of *D& ( on testing for *D&, D-dimer). Increased levels of *D& ( on testing for *D&, D-dimer)
+. Schistocytes on peripheral smear+. Schistocytes on peripheral smear
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TREATMENTTREATMENT%. Individual treatment%. Individual treatment
- reatment closely !ith the etiology, ages, and hemodinamic situation.- reatment closely !ith the etiology, ages, and hemodinamic situation.
- Determine the underlying cause of the patients DIC and initiate- Determine the underlying cause of the patients DIC and initiatetherapytherapy
'. eneral$'. eneral$
a.a. /onitor vital signs and attend to life-threatening issues such as air!ay/onitor vital signs and attend to life-threatening issues such as air!ay compromisecompromise
b. Stop the coagulation $b. Stop the coagulation $
#xp$ "eparin, give if $#xp$ "eparin, give if $
- &rimary disease can0t be stop 1uic2ly- &rimary disease can0t be stop 1uic2ly
- 3ith ischemia or thromboembolism- 3ith ischemia or thromboembolism c. Substitution treatmentc. Substitution treatment
transfusions of fresh fro4en plasma or platelet.transfusions of fresh fro4en plasma or platelet.
d. 5sam trane2samat as antifibrinolysis if indicatedd. 5sam trane2samat as antifibrinolysis if indicated
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Henoch SchoeleinHenoch Schoelein
Syndo!eSyndo!e
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1enoch 6 Scholein1enoch 6 Scholein
SyndromeSyndrome0n abnormal inAamation process0n abnormal inAamation processthat involve a peripheral vasulerthat involve a peripheral vasulerwall especially at sin, digestivuswall especially at sin, digestivus
trac, idney and #oints.trac, idney and #oints. nsidens :nsidens :
+hildren, F I years old+hildren, F I years old
"ale : >emale B F : E"ale : >emale B F : E "ore incidence happen at the spring"ore incidence happen at the spring
and winterand winter
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EtiologyEtiology
diopatidiopati
mmune reaction g0mmune reaction g0
nfection )irus or baterianfection )irus or bateria HerediterHerediter
'rugs allergy'rugs allergy
nsect bittingnsect bitting >ood>ood
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Clinical *aniestationClinical *aniestation
ash Purpura 8ex : Petechiae ash Purpura 8ex : Petechiae
0bdominal pain0bdominal pain
Poliatralgia 7 Joint swellingPoliatralgia 7 Joint swelling ?astrointestinal bleeding?astrointestinal bleeding
enal = -idney 'iseaseenal = -idney 'isease
+1/ manifestation+1/ manifestation
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7atomechanism7atomechanism
!tiology of H//!tiology of H//
ncreased production of g0ncreased production of g0
g0 spreading out and gathered at theg0 spreading out and gathered at theperipehral vasuler wallperipehral vasuler wall
'isturbing and 0ltering the vasular'isturbing and 0ltering the vasularendotel cell main functionendotel cell main function
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7atomechanism7atomechanism
!ndotel cell stimulates chemical mediator,!ndotel cell stimulates chemical mediator,
such as *1> alfa and 3 Isuch as *1> alfa and 3 I
ncreased of an abnormal apoptosis ofncreased of an abnormal apoptosis ofendotelial cellendotelial cell
1eutrol and limfosit inltration1eutrol and limfosit inltration
nAamationnAamation
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Supporti!e )estSupporti!e )est
3aboratorium :3aboratorium : +omplete lood +ount 3euocytosis+omplete lood +ount 3euocytosis
9rinalysis Hematuria 7 proteinuria9rinalysis Hematuria 7 proteinuria
g0 serum level increasedg0 serum level increased /in iopsy 3euocytoclastic vasulitis/in iopsy 3euocytoclastic vasulitis
adiographyadiography
+hest and abdominal maging+hest and abdominal maging
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)herapy)herapy
ed rest 7 /upportive careed rest 7 /upportive care
/elf 3imiting disease/elf 3imiting disease
8resolve in 6 I wees8resolve in 6 I wees
mmunosuppresan +yclophosphamidemmunosuppresan +yclophosphamide
+orticosteroid+orticosteroid
1/0' 81on /teroid 0nti nAamation 'rugs1/0' 81on /teroid 0nti nAamation 'rugs; 'apsone'apsone
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IdiopathicIdiopathic
)rombocytopenic)rombocytopenic7urpura7urpura
8I)798I)79leeding, petechie = eimosis onleeding, petechie = eimosis onthe sin or mucous membranethe sin or mucous membrane
with trombocyte reduction whichwith trombocyte reduction whichis usually idiopathic.is usually idiopathic.
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Classi:cationClassi:cation
0cute0cute : children F;I years old,: children F;I years old,
I5 KLMI5 KLM
+hronic+hronic : adults E5;5L years: adults E5;5L years
old, EL E5Mold, EL E5M
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Acute I)7Acute I)7
ncidence ;D age : F I years oldncidence ;D age : F I years old
>emale D "ale B N : G or F : E>emale D "ale B N : G or F : E
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7atomechanism7atomechanism
0utoantibody g? binding on the platelet surface0utoantibody g? binding on the platelet surface
Platelet sensitatedPlatelet sensitated
"acrofage !/ fagocytosis"acrofage !/ fagocytosis
8 in hepar and spleen8 in hepar and spleen
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Signs and SymptomsSigns and Symptoms
leeding ;D sin and mucousleeding ;D sin and mucousmembrane 8petechie, melena,membrane 8petechie, melena,epitasis, hematuri, cerebralepitasis, hematuri, cerebral
bleeding at severe trombocytopeni.bleeding at severe trombocytopeni. *iredness, weight loss, limph gland*iredness, weight loss, limph gland
enlargementenlargement
>eeling of saturated stomach,>eeling of saturated stomach,stomach painstomach pain
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)ests and Examinations)ests and Examinations
Peripheral blood test B trombocytopenia,Peripheral blood test B trombocytopenia,normocytic, normal leucocyte andnormocytic, normal leucocyte andascending on heavy bleedingascending on heavy bleeding
one marrow test B commonly normal,one marrow test B commonly normal,increasing of megaariocyteincreasing of megaariocyte
eduction of coagulation retractioneduction of coagulation retraction
!xtended bleeding time!xtended bleeding time
/horter P+*/horter P+*
*urniet *est 87*urniet *est 87
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)reatment)reatment
/ome patients ;D /pontaneous/ome patients ;D /pontaneous
remisionremision
/evere ;D corticosteroid/evere ;D corticosteroid
ntravenous heparinntravenous heparin
*ransfusion of trombocyte*ransfusion of trombocyte
suspensionsuspension
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EthiologyEthiology
diopathicdiopathic
Hipersplenism, viral infection, food,Hipersplenism, viral infection, food,
'rugs : asetosal, paraaminosalisilat,'rugs : asetosal, paraaminosalisilat,fenulbutasol, diamos, ina, sidermitfenulbutasol, diamos, ina, sidermit
+hemical materials+hemical materials
"alnutrition ;D lac of maturation"alnutrition ;D lac of maturationfactorfactor
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