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HEMOSTATIC MECHANISM

The classic mechanism include :

1. Tissue factor

2. Vascular response3. Platelet adhesion

platelet aggregation

4. Clot formation

clot stabilizationlimitation of clotting

5. Fibrinolysis


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NORMAL HAEMOSTASIS

VESSEL INJURY

COLLAGEN EXPOSURE

PLATELET RELEASE

REACTION

TROMBOXANE A2, ADP

PLATELET AGGREGATION

PRIMARY HAEMOSTATIC PLUG

PLATELET FUSION

STABLE HEMOSTATIC PLUG FIBRIN

REDUCED

BLOOD FLOW THROMBIN

BLOOD

COAGULATIONVACOKONSTRICTION

SEROTONINPLATELET

FACTORS

F. XII

TISSUE

TROMBOPLASTIN

4NORMAL HEMOSTASISVESSEL INJURY

TISSUETROMBOPLASTI


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44Clotting CascadeClotting Cascade


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55Hemostatic mechanismHemostatic mechanism


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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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2.2. UNCOMMON INHERITED DEFICIENCIESUNCOMMON INHERITED DEFICIENCIES

-- FIBRINOGEN & DYSFIBRINOGENFIBRINOGEN & DYSFIBRINOGEN-- PROTHROMBRIN (F.II) &PROTHROMBRIN (F.II) &

DYSPROTHOMBRINDYSPROTHOMBRIN

-- F. V ( PARAHEMOPHILIA )F. V ( PARAHEMOPHILIA )-- F. VIIF. VII

-- F. XF. X

-- F.XIIF.XII

-- F.XIIIF.XIII

-- COMBINED DEFICIENCIESCOMBINED DEFICIENCIES

-- OTHEROTHER

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3.3. ACQUIRED DEFICIENCIESACQUIRED DEFICIENCIES

-- VIT. KVIT. K

-- CONSUMPTION COAGULOPATHYCONSUMPTION COAGULOPATHY

-- PARENCHYMAL LIVER DISEASEPARENCHYMAL LIVER DISEASE

-- CONGENITAL HEART DISEASECONGENITAL HEART DISEASE-- CARDIOPULMONARY BYPASSCARDIOPULMONARY BYPASS

-- RENAL DISEASERENAL DISEASE

-- ACQUIRED INHIBITORSACQUIRED INHIBITORS( CIRCULATING ANTICOAGULANTS )( CIRCULATING ANTICOAGULANTS )

-- OTHEROTHER

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4.4. NEONATAL DEFICIENCIESNEONATAL DEFICIENCIES

-- HEMORRHAGIC DISEASE OF THEHEMORRHAGIC DISEASE OF THE

NEWBORNNEWBORN-- INHERITED DEFICIENCIESINHERITED DEFICIENCIES

-- ACQUIRED DEFICIENCIESACQUIRED DEFICIENCIES

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Factor VIII or Factor IX deficiencyFactor VIII or Factor IX deficiency

( Hemophilia A or B )( Hemophilia A or B )

Hemophilia occurs in 1 : 5000 males

with 85% having factor VIII deficiency

and 10 15% having factor IX deficiency

Clinical manisfestationsClinical manisfestations

Bleeding present from birth or

Occur in the fetus

Intracranial hemorrhage neonates30 % male infant bleed with circumcision

Intramuscular hematomas

Hemarthroses hallmark


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Laboratory findingLaboratory finding

Reduce level of factor VIII or IXReduce level of factor VIII or IX

APTT is two to three timesAPTT is two to three times

Platelet countPlatelet count

Bleeding timeBleeding time

Prothrombin timeProthrombin time

Thrombin timeThrombin time

Normal


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Genetics and classificationGenetics and classification

No apparent racial and ethnic groupNo apparent racial and ethnic group

XX--Linked traits.Linked traits.

Severe hemophilia < 1% U/dl clotting factorSevere hemophilia < 1% U/dl clotting factor

Bleeding spontaneousBleeding spontaneous

11 5% U/dl5% U/dl

Mild traumaMild trauma induce bleedinginduce bleeding

Mild hemophilia > 5% U/dlMild hemophilia > 5% U/dl

Significant traumaSignificant trauma bleedingbleeding


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TreatmentTreatment

The prevention of traumaThe prevention of trauma Aspirin and NSAD should be avoidedAspirin and NSAD should be avoided

Psychosocial interventionPsychosocial intervention

Immunized hepatitis BImmunized hepatitis B Bleeding occurs level factor VIII or IXBleeding occurs level factor VIII or IX

must bemust be

raised 35raised 35 40 U/dl or40 U/dl orfor life threatening to 100 U/dlfor life threatening to 100 U/dl


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GUIDELINES FOR REPLACEMENTGUIDELINES FOR REPLACEMENTTHERAPY BEFORE AND AFTERTHERAPY BEFORE AND AFTER

ELECTIVE SURGERY:ELECTIVE SURGERY:

A. BEFORE PROCEDURE:A. BEFORE PROCEDURE:

1. COMPLETE COAGULATION1. COMPLETE COAGULATIONWORKUPWORKUP

2. INCUBATED TEST FOR2. INCUBATED TEST FORINHIBITORSINHIBITORS

3. CALCULATE NEEDS &3. CALCULATE NEEDS &STOCKPILE THERAPEUTICSTOCKPILE THERAPEUTIC

MATERIALMATERIAL

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4. DETERMINE HALF4. DETERMINE HALF--LIFE OFLIFE OF

THERAPEUTIC MATERIALTHERAPEUTIC MATERIAL

5. COMPLETE BLOOD, PLATELET,5. COMPLETE BLOOD, PLATELET,AND RETICULOCYTE COUNTSAND RETICULOCYTE COUNTS

6. DETERMINE RED CELL TYPE6. DETERMINE RED CELL TYPE

B. GENERAL SURGICAL PROCEDURE:B. GENERAL SURGICAL PROCEDURE:

1. MINOR1. MINOR

A. GIVE DOSE CALCULATED TOA. GIVE DOSE CALCULATED TO

BRING PATIENTS PLASMABRING PATIENTS PLASMALEVEL TO 100 % 1 HOURLEVEL TO 100 % 1 HOUR

BEFORE PROCEDURE (50BEFORE PROCEDURE (50 UNITS/KG)UNITS/KG)

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B. MAINTAIN PLASMA LEVELB. MAINTAIN PLASMA LEVELABOVE 60 % FOR 4 DAYSABOVE 60 % FOR 4 DAYS

C. MAINTAIN PLASMA LEVELC. MAINTAIN PLASMA LEVELABOVE 20 % FOR SUBSEQUENTABOVE 20 % FOR SUBSEQUENT4 DAYS4 DAYS

D. ASSAY DAILY BEFORED. ASSAY DAILY BEFOREADMINISTRATIONADMINISTRATION

2. MAJOR2. MAJOR

A. GIVE DOSE CALCULATED TOA. GIVE DOSE CALCULATED TOBRING PATIENTS PLASMABRING PATIENTS PLASMALEVEL TO 100 % 1 HOUR BEFORELEVEL TO 100 % 1 HOUR BEFOREPROCEDURE ( 50 UNITS/KG )PROCEDURE ( 50 UNITS/KG )

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2. MAINTAIN PLASMA LEVEL2. MAINTAIN PLASMA LEVEL

ABOVE 80 % FOR 4 DAYSABOVE 80 % FOR 4 DAYS

( 40 UNITS/KG 3 TIMES DAILY )( 40 UNITS/KG 3 TIMES DAILY )

3. MAINTAIN PLASMA LEVEL3. MAINTAIN PLASMA LEVEL

ABOVE 40 % FOR SUBSEQUENT 4ABOVE 40 % FOR SUBSEQUENT 4

DAYS (40 UNITS/KG 2 TIMES DAILY )DAYS (40 UNITS/KG 2 TIMES DAILY )4. ASSAY DAILY BEFORE4. ASSAY DAILY BEFORE

ADMINISTRATIONADMINISTRATION

5. IF PATIENT IS IN CAST,5. IF PATIENT IS IN CAST,DISCONTINUE REPLACEMENTDISCONTINUE REPLACEMENT

UNTIL REHABILITATION PROGRAMUNTIL REHABILITATION PROGRAM

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6. IF PATIENT IS NOT IN CAST,6. IF PATIENT IS NOT IN CAST,MAINTAIN PLASMA LEVEL ABOVEMAINTAIN PLASMA LEVEL ABOVE

20 % FOR AMBULATION20 % FOR AMBULATION7. FOR REHABILITATION PROGRAM7. FOR REHABILITATION PROGRAM

MAINTAIN PLASMA LEVEL ABOVEMAINTAIN PLASMA LEVEL ABOVE10 % FOR 3 WEEKS10 % FOR 3 WEEKS

D. DENTAL PROCEDURE:D. DENTAL PROCEDURE:

1. GIVE EACA 100 MG/KG 4 HOURS1. GIVE EACA 100 MG/KG 4 HOURSBEFORE SURGERYBEFORE SURGERY

2. GIVE CLOTTING FACTOR DOSE2. GIVE CLOTTING FACTOR DOSECALCULATED TO BRING PLASMACALCULATED TO BRING PLASMALEVEL TO 100 % 1 HOUR BEFORELEVEL TO 100 % 1 HOUR BEFOREPROCEDUREPROCEDURE

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3. CONTINUE EACA 1

00MG/KG

3. CONTINUE EACA 1

00MG/KGEVERY 6 HOURS FOR 10 TO 14 DAYSEVERY 6 HOURS FOR 10 TO 14 DAYS

4. REPEAT REPLACEMENT THERAPY4. REPEAT REPLACEMENT THERAPY

IN 3 DAYS IF PROCEDURE ISIN 3 DAYS IF PROCEDURE IS

EXTENSIVEEXTENSIVE

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TreatmentTreatment

There is no concentrate factor XIThere is no concentrate factor XI

Fresh Frozen Plasma (FFP)Fresh Frozen Plasma (FFP)

Minor surgeryMinor surgery local pressurelocal pressure

Plasma infusion 1U/kgPlasma infusion 1U/kg increaseincreaseplasmaplasma

concentration by 2 U/dlconcentration by 2 U/dl

The infusion of 10The infusion of 10 --15ml/kg plasma15ml/kg plasma sufficient to control moderatesufficient to control moderatehemorrhagehemorrhage


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von Willebrand Diseasevon Willebrand Disease

Hereditary bleeding disorderHereditary bleeding disorder

11 2% of general population2% of general population

Inherited autosomalInherited autosomal

Women > menWomen > men

Classified : type 1, 2 and 3Classified : type 1, 2 and 3


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TreatmentTreatment

Increasing the plasma level of VWF andIncreasing the plasma level of VWF and

Factor VIIIFactor VIII

Desmopressin (DDAVP)Desmopressin (DDAVP)

Plasma derived VWF :Plasma derived VWF : 1 U/kg1 U/kg oo1.5 U/dl1.5 U/dl

Purified VWF concentratePurified VWF concentrate futurefuture

-- presurgical management orpresurgical management or

prophylaxisprophylaxis


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DICDIC

Disseminated intravascular coagulationDisseminated intravascular coagulation

(Consumptive Coagulopathy )(Consumptive Coagulopathy )

Consumption of clotting factors, plateletsConsumption of clotting factors, platelets

and anticoagulant proteinand anticoagulant protein


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Clinical manifestationClinical manifestation

Bleeding from site of venipunctureBleeding from site of venipuncture

Petechiae and ecchymosesPetechiae and ecchymoses

LaboratoryLaboratory PT, APTT, TTPT, APTT, TT oo

Platelet countPlatelet count

FDPFDP oo Hemolytic anemiaHemolytic anemia


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TreatmentTreatment

Identification and treatment of theIdentification and treatment of the

triggering eventtriggering event

Replacement therapyReplacement therapy

-- PLATELET CONCENTRATES ( 1U/10 KG)PLATELET CONCENTRATES ( 1U/10 KG)

-- CRYOPRECIPIATE ( 50CRYOPRECIPIATE ( 50--100 MG/KG100 MG/KG

FIBRINOGEN )FIBRINOGEN )

-- FRESHFRESH--FROZEN PLASMA ( 10FROZEN PLASMA ( 10--1515 ML/KG,ML/KG,INITIALLY ; MAY NEED 5 ML/KG/6JAM)INITIALLY ; MAY NEED 5 ML/KG/6JAM)

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INTRAVENOUS HEPARINIZATION:INTRAVENOUS HEPARINIZATION:

-- INTERMITTEN SCHEDULE :INTERMITTEN SCHEDULE :

100 U/KG/4 hours100 U/KG/4 hours

-- CONTINUOUS SCHEDULE :CONTINUOUS SCHEDULE :

50 U/KG INITIAL BOLUS, than50 U/KG INITIAL BOLUS, than

25 U/KG/hours with CONTINUOUS25 U/KG/hours with CONTINUOUSINFUSONINFUSON

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