Kuliah

Hemostasis

1
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

3
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

6
 7
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
 8
3. ACQUIRED DEFICIENCIES
- VIT. K
- CONSUMPTION COAGULOPATHY
- PARENCHYMAL LIVER DISEASE
- CONGENITAL HEART DISEASE
- CARDIOPULMONARY BYPASS
- RENAL DISEASE
- ACQUIRED INHIBITORS
( CIRCULATING ANTICOAGULANTS )
- OTHER
8
 9

4. NEONATAL DEFICIENCIES
- HEMORRHAGIC DISEASE OF THE
NEWBORN
- INHERITED DEFICIENCIES
- ACQUIRED DEFICIENCIES

9
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

11
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

12
 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

13
GUIDELINES FOR REPLACEMENT 5
THERAPY BEFORE AND AFTER
ELECTIVE SURGERY:

A. BEFORE PROCEDURE:
1. complete coagulation workup
2. incubated test for inhibitors
3. calculate needs &
stockpile therapeutic material

14
4. determine half-life of therapeutic material 6
5. complete blood, platelet, and reticulocyte counts
6. determine red cell type

B. GENERAL SURGICAL PROCEDURE:

1. MINOR
A. give dose calculated to bring
patient’s
plasma level to 100 % 1 hour
before procedure (50 Units/kg BW)

15
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
patient’s plasma level to 100 % 1
hour before procedure ( 50 units/kg )
16
 8
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
PATIENT’S PLASMA LEVEL TO
100 % 1 HOUR BEFORE
PROCEDURE ( 50 UNITS/KG )
17
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
18
6. IF PATIENT IS NOT IN CAST,10
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
 11

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

20
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

21
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
22
von Willebrand Disease

 Hereditary bleeding disorder

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

23
Clinical manifestations

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

Laboratory findings

Bleeding time 
PTT 
Platelet count was normal
24
25
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

26
DIC
Disseminated intravascular coagulation
(Consumptive Coagulopathy )

Consumption of clotting factors, platelets

and anticoagulant protein

27
28
29
30
 Clinical manifestation

 Bleeding from site of venipuncture

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

31
 6
Treatment
 Identification and 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)
32
 7
INTRAVENOUS HEPARINIZATION:

- INTERMITTEN SCHEDULE :
100 U/KG/4 hours

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

33
34