Download - Hemophilia Powerpoint-Kuliah FKIK UNTAN
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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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