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HEMOSTATIC MECHANISM

The classic mechanism include :


1. Tissue factor
2. Vascular response
3. Platelet adhesion
platelet aggregation
4. Clot formation
clot stabilization
limitation of clotting
5. Fibrinolysis
NORMAL
NORMALHAEMOSTASIS
HEMOSTASIS 4
VESSEL
VESSELINJURY
INJURY
TISSUE
TISSUE
COLLAGEN EXPOSURE TROMBOPLASTIN
TROMBOPLASTIN
F. XII
PLATELET RELEASE
REACTION
SEROTONIN PLATELET
FACTORS
BLOOD
VACOKONSTRICTION TROMBOXANE A2, ADP COAGULATION
REDUCED PLATELET AGGREGATION
BLOOD FLOW THROMBIN
PRIMARY HAEMOSTATIC PLUG

PLATELET FUSION

STABLE HEMOSTATIC PLUG FIBRIN


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Clotting Cascade 4
Hemostatic mechanism 5
Hereditary Clotting factor Deficiencies
( Bleeding Disorders )

1. COMMON INHERITED DEFICIENCY


- F. VIII HEMOPHILIA A
- vWB Von Willebrand Diasese
- F. IX HEMOPHILIA B
- F. XI HEMOPHILIA C

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2. UNCOMMON INHERITED DEFICIENCIES
- FIBRINOGEN & DYSFIBRINOGEN
- PROTHROMBRIN (F.II) &
DYSPROTHOMBRIN
- F. V ( PARAHEMOPHILIA )
- F. VII
- F. X
- F.XII
- F.XIII
- COMBINED DEFICIENCIES
- OTHER 7
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3. ACQUIRED DEFICIENCIES
- VIT. K
- CONSUMPTION COAGULOPATHY
- PARENCHYMAL LIVER DISEASE
- CONGENITAL HEART DISEASE
- CARDIOPULMONARY BYPASS
- RENAL DISEASE
- ACQUIRED INHIBITORS
( CIRCULATING ANTICOAGULANTS )
- OTHER
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4. NEONATAL DEFICIENCIES
- HEMORRHAGIC DISEASE OF THE
NEWBORN
- INHERITED DEFICIENCIES
- ACQUIRED DEFICIENCIES

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Factor VIII or Factor IX deficiency
( Hemophilia A or B )

Hemophilia occurs in 1 : 5000 males


with 85% having factor VIII deficiency
and 10 15% having factor IX deficiency

Clinical manisfestations
Bleeding present from birth or
Occur in the fetus
Intracranial hemorrhage neonates
30 % male infant bleed with circumcision
Intramuscular hematomas
Hemarthroses hallmark 10
Laboratory finding

Reduce level of factor VIII or IX


APTT is two to three times
Platelet count
Bleeding time
Normal
Prothrombin time
Thrombin time

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Genetics and classification

No apparent racial and ethnic group


X-Linked traits.

Severe hemophilia < 1% U/dl clotting factor


Bleeding spontaneous

1 5% U/dl
Mild trauma induce bleeding

Mild hemophilia > 5% U/dl


Significant trauma bleeding

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Treatment
The prevention of trauma
Aspirin and NSAD should be avoided
Psychosocial intervention
Immunized hepatitis B
Bleeding occurs level factor VIII or IX
must be
raised 35 40 U/dl or
for life threatening to 100 U/dl

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GUIDELINES FOR REPLACEMENT
THERAPY BEFORE AND AFTER
ELECTIVE SURGERY:

A. BEFORE PROCEDURE:
1. COMPLETE COAGULATION
WORKUP
2. INCUBATED TEST FOR
INHIBITORS
3. CALCULATE NEEDS &
STOCKPILE THERAPEUTIC
MATERIAL
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4. DETERMINE HALF-LIFE OF
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THERAPEUTIC MATERIAL
5. COMPLETE BLOOD, PLATELET,
AND RETICULOCYTE COUNTS
6. DETERMINE RED CELL TYPE

B. GENERAL SURGICAL PROCEDURE:


1. MINOR
A. GIVE DOSE CALCULATED TO
BRING PATIENTS PLASMA
LEVEL TO 100 % 1 HOUR
BEFORE PROCEDURE (50 UNITS/KG)
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B. MAINTAIN PLASMA LEVEL 7
ABOVE 60 % FOR 4 DAYS
C. MAINTAIN PLASMA LEVEL
ABOVE 20 % FOR SUBSEQUENT
4 DAYS
D. ASSAY DAILY BEFORE
ADMINISTRATION

2. MAJOR
A. GIVE DOSE CALCULATED TO
BRING PATIENTS PLASMA
LEVEL TO 100 % 1 HOUR BEFORE
PROCEDURE ( 50 UNITS/KG ) 16
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B. MAINTAIN PLASMA LEVEL ABOVE
60 % FOR 4 DAYS
C. MAINTAIN PLASMA LEVEL ABOVE
40 % FOR NEXT 4 DAYS OR UNTIL
ALL DRAINS & SUTURES REMOVED
D. ASSAY DAILY BEFORE
ADMINISTRATION
C. ORTHOPEDIC PROCEDURE:
1. GIVE DOSE CALCULATED TO
BRING PATIENTS PLASMA LEVEL TO
100 % 1 HOUR BEFORE
PROCEDURE ( 50 UNITS/KG )
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2. MAINTAIN PLASMA LEVEL 9
ABOVE 80 % FOR 4 DAYS
( 40 UNITS/KG 3 TIMES DAILY )
3. MAINTAIN PLASMA LEVEL
ABOVE 40 % FOR SUBSEQUENT 4
DAYS (40 UNITS/KG 2 TIMES DAILY )
4. ASSAY DAILY BEFORE
ADMINISTRATION
5. IF PATIENT IS IN CAST,
DISCONTINUE REPLACEMENT
UNTIL REHABILITATION PROGRAM
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6. IF PATIENT IS NOT IN CAST, MAINTAIN
PLASMA LEVEL ABOVE 20 % FOR
AMBULATION
7. FOR REHABILITATION PROGRAM
MAINTAIN PLASMA LEVEL ABOVE 10 %
FOR 3 WEEKS
D. DENTAL PROCEDURE:
1. GIVE EACA 100 MG/KG 4 HOURS
BEFORE SURGERY
2. GIVE CLOTTING FACTOR DOSE
CALCULATED TO BRING PLASMA
LEVEL TO 100 % 1 HOUR BEFORE
PROCEDURE 19
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3. CONTINUE EACA 100 MG/KG


EVERY 6 HOURS FOR 10 TO 14 DAYS
4. REPEAT REPLACEMENT THERAPY
IN 3 DAYS IF PROCEDURE IS
EXTENSIVE

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Factor XI Deficiency
( Hemophilia C )

An autosomal deficiency
Mild and moderate bleeding symptoms
In Israel 1-3/1000 are homozygous
The bleeding not correlated with the amount of

Factor XI
Severe deficiency minimal or no symptoms

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Treatment

There is no concentrate factor XI


Fresh Frozen Plasma (FFP)
Minor surgery local pressure
Plasma infusion 1U/kg increase
plasma
concentration by 2 U/dl
The infusion of 10 -15ml/kg plasma
sufficient to control moderate
hemorrhage
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von Willebrand Disease

Hereditary bleeding disorder


1 2% of general population
Inherited autosomal
Women > men
Classified : type 1, 2 and 3

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Clinical manifestations

Mucocutaneous hemorrhage
- Excessive bruising, epistaxis, menorrhagia
- Post op hemorrhage

Laboratory findings

Bleeding time
PTT
Platelet count was normal
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Treatment

Increasing the plasma level of VWF and


Factor VIII
Desmopressin (DDAVP)
Plasma derived VWF : 1 U/kg 1.5
U/dl
Purified VWF concentrate future

- presurgical management or
prophylaxis

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DIC
Disseminated intravascular coagulation
(Consumptive Coagulopathy )

Consumption of clotting factors, platelets


and anticoagulant protein

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Clinical manifestation

Bleeding from site of venipuncture


Petechiae and ecchymoses
Laboratory
PT, APTT, TT
Platelet count
FDP
Hemolytic anemia

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Treatment
Identificationand treatment of the
triggering event
Replacement therapy

- PLATELET CONCENTRATES ( 1U/10 KG)


- CRYOPRECIPIATE ( 50-100 MG/KG
FIBRINOGEN )
- FRESH-FROZEN PLASMA ( 10-15 ML/KG,
INITIALLY ; MAY NEED 5
ML/KG/6JAM)
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INTRAVENOUS HEPARINIZATION:

- INTERMITTEN SCHEDULE :
100 U/KG/4 hours

- CONTINUOUS SCHEDULE :
50 U/KG INITIAL BOLUS, than
25 U/KG/hours with CONTINUOUS
INFUSON

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