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HEMOPHILIADr. Rini Andriani, Sp.AFKIK UNTAN8 Juni 20111Bleeding/ Easy Bruisability

Low Platelet countNormal Platelet Count

Platelet MorphologyPT/aPTT

Small Platelet Large Platelet Anemia No anemia Dec retics Normal retics

Aplastic anemia ITPLeukemiaMegaloblastic anemia

2PT/aPTT

Normal PT Normal PT Prolonged PT Prolonged PT Normal PTT Prolonged PTT Normal PTT Prolonged PTT

Platelet 1:1 mixing studies Vit K DeficiencyLiver DiseaseDysfunction vWB Disease Factor VII Def Vit K DefvWB Dse DIC3 1:1 Mixing Studies

Normal PTTProlonged PTT

Factor VIII/IX Deficiency Inhibitors

Specific Factor Assay

4FINDINGS HEMOPHILIA HEMOPHILIA vWB A B DISESASE

Bleeding Time Normal Normal Prolonged

PT Normal NormalNormal

aPTT Prolonged Prolonged Prolonged

Factor VIII Low Normal Low/Normal

Factor IX Normal LowNormal

vWB Factor Normal Normal Low5FINDINGSHEMOPHILIAvWB DISEASESymptomsJoint & muscle bleedingBruising, epistaxis & mucosal bleedGender dist.Males>FemalesMale=FemaleFrequency1:60001:200-500Abn CHONFactor VIIIvWB FactorBleeding timeNormalProlongedPTTProlongedNormal/ProlongedFactor VIIIDec to absentBorderline to dec6What is Hemophilia?It is an inherited disorder of blood clotting7HEMOSTASISPRIMARY HEMOSTASIS=primary clot (platelet)

SECONDARY HEMOSTASIS = coagulation factors8CLOTTING CASCADEIntrinsic PathwayExtrinsic Pathway ContactXIIXIIa

XIXIa Tissue Factor

IX IXaVIIaVII VIIIa+Pl XXa Va+PlII IIa

Fibrinogen Fibrin Clot9Clotting is a complex process in which the activated form of one coagulation factor activates the next factor in a clotting sequence

10How does bleeding stop?VasoconstrictionPlatelet plug formationFibrin clot

In a person with hemophilia, the clot does not form properly due to lack of clotting factor

11The things in the blood that stop bleeding are called clotting factors.

These clotting factors stop bleeding quickly in people who do not have haemophilia.

When we bleed, a tear is made in a blood pathway. Right away, the tube reacts by tightening up and special blood cells begin to close the hole. Then the clotting factors start to work to knit a cover for the hole.

People who have haemophilia are missing one of these clotting factors, or one of them does not work properly. As a result, no cover is made for the hole. The things in the blood that stop bleeding are called clotting factors.

These clotting factors stop bleeding quickly in people who do not have haemophilia.

When we bleed, a tear is made in a blood pathway. Right away, the tube reacts by tightening up and special blood cells begin to close the hole. Then the clotting factors start to work to knit a cover for the hole.

People who have haemophilia are missing one of these clotting factors, or one of them does not work properly. As a result, no cover is made for the hole. The things in the blood that stop bleeding are called clotting factors.

These clotting factors stop bleeding quickly in people who do not have haemophilia.

When we bleed, a tear is made in a blood pathway. Right away, the tube reacts by tightening up and special blood cells begin to close the hole. Then the clotting factors start to work to knit a cover for the hole.

People who have haemophilia are missing one of these clotting factors, or one of them does not work properly. As a result, no cover is made for the hole. . How does bleeding stop? The things in the blood that stop bleeding are called clotting factors.

These clotting factors stop bleeding quickly in people who do not have haemophilia.

When we bleed, a tear is made in a blood pathway. Right away, the tube reacts by tightening up and special blood cells begin to close the hole. Then the clotting factors start to work to knit a cover for the hole.

People who have haemophilia are missing one of these clotting factors, or one of them does not work properly. As a result, no cover is made for the hole. . How does bleeding stop? The things in the blood that stop bleeding are called clotting factors.

These clotting factors stop bleeding quickly in people who do not have haemophilia.

When we bleed, a tear is made in a blood pathway. Right away, the tube reacts by tightening up and special blood cells begin to close the hole. Then the clotting factors start to work to knit a cover for the hole.

People who have haemophilia are missing one of these clotting factors, or one of them does not work properly. As a result, no cover is made for the hole. . How does bleeding stop? The things in the blood that stop bleeding are called clotting factors.

These clotting factors stop bleeding quickly in people who do not have haemophilia.

When we bleed, a tear is made in a blood pathway. Right away, the tube reacts by tightening up and special blood cells begin to close the hole. Then the clotting factors start to work to knit a cover for the hole.

People who have haemophilia are missing one of these clotting factors, or one of them does not work properly. As a result, no cover is made for the hole. . How does bleeding stop? The things in the blood that stop bleeding are called clotting factors.

These clotting factors stop bleeding quickly in people who do not have haemophilia.

When we bleed, a tear is made in a blood pathway. Right away, the tube reacts by tightening up and special blood cells begin to close the hole. Then the clotting factors start to work to knit a cover for the hole.

People who have haemophilia are missing one of these clotting factors, or one of them does not work properly. As a result, no cover is made for the hole.

HemophiliaA person with hemophilia has less clotting factor than usual.Hemophilia A deficiency of factor VIIIHemophilia B deficiency of factor IX

Normal range of factor activity: 50 200%HemophiliaSevere: less than 1%Moderate: 1 5%Mild: 6 30%12FACTOR LEVEL AND SEVERITY OF CLINICAL MANIFESTATIONS TYPE PERCENTMANIFESTATION Factor VIII

Severe 5 minutesApply iceAvoid IM injections unless administered under replacementVenipuncture or superficial vein is safe if pressure is applied for several minutesFemoral or jugular venipuncture should not be attempted.28General CareExercise Encourage participation in sensible, physical exercise because strong muscles help support joints. Ideal exercises: swimming & bicycling- strengthen muscles and preserve normal joint motion Avoid jogging and contact sports.29Advances in ManagementFactor ConcentratesHome TreatmentProphylactic Care/Port-a-cathContinuous InfusionProducts Used to treat patients with Inhibitors/ImmunosuppressionComprehensive Hemophilia Care30Thank You!31CLOTTING CASCADEPTTPTContact Factors

IIaVIIaTENETFibrinogenFibrinXL FIBRINXIIITF32

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