Download - Approach to a Bleeding Disease
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APPROACH TO A BLEEDING DISEASE
Presented by-DaminiB.Sc-M.L.T-3rd yearModerator-Dr.Anju
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Investigation of a suspected bleeding tendency may begin from 3 diff. points:
1. Investigating a clinically suspected bleeding disease.If the bleeding history or family history is significant,appropriate tests should be performed
2. Follow-up of an abnormal first line test3. Investigation of acute haemostatic failure
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• Bleeding disorder result from abormality of vessel wall, platelets or coagulation factors
• Evaluation of a patient with bleeding disorder can be divided into two parts:-
₁ Clinical evaluation :a. Historyb. Physical examinationc. Family history₂ Laboratory evaluation:a. Screening testsb. Specific tests
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Clues to congenital disorders
• Excessive bleeding,by common childhood trauma
• History of bleeding disorder in family
Clues to an acquired disorder• Patient presents with bleeding in recent past• Exposure to potentially causative factors and
onset of bleeding episodes.
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Nature of bleeding• Sites of bleeding may suggest where in the
coagulation defect may be seen e.g:-
mucous membrane bleeding and petechiae are seen in platelet disorders
haemarthrosis is common in haemophilia
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• Severity of bleeding
bleeding after major surgery or trauma suggests only a mild bleeding disorder
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• Medications,critical in evaluation of coagulation abnormality
( I) Aspirin leading to platelet abnormality (2) Extensive use of drugs known to induce thrombocytopenia (3) Acquired factor inhibitors associated
with medications like penicillin
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Laboratory Approach
1. Demonstration of the defect2. Identification of the defect(s)3. Assessment of severity4. Consequential studies eg. carrier detection5. Monitoring of treatment
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Screening Tests
1. Platelet count & morphology 2. Bleeding Time 3. Clotting Time 4. Prothrombin Time 5. Activated Partial Thromboplastin Time
6. Thrombin Time
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Investigation of vascular disorders
• Vascular disorders are those which arise due to defect or deficiency of the vessel wall
• Investigation to be carried out is the BLEEDING TIME
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Investigation of a platelet function disorder
• The peripheral blood platelet count and bleeding time are the first line tests of platelet function disorder
• Platelet function tests can be divided into :
ADHESION TESTS• Retention in glass bead column• Baumgartner’s technique
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AGGREGATION TESTS• Turbidometric technique using
ADP,collagen,ristocetin,adrenaline,thrombin,arac-hidonic acid
INVESTIGATION OF GRANULAR CONTENT AND RELEASE
• Electron microscopy – dense bodies• Beta-thromboglobulin • Platelet factor 4 granules• vWF
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PROSTAGLANDIN PATHWAYS• radio immuno assay• Studies with radioactive arachidonic acid• MDA assay using thiobarbituric acid
PLATELET COAGULANT ACTIVITY• Prothrombin consumption index
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Investigations of coagulation factor defect
• An individual may have a deficiency of a coagulation factor because of a variant of the molecule is synthesized which is deficient in clotting activity
• When the screening tests indicate that an individual has a coagulation defect,the plasma conc. of the coagulation factor should be assayed
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Investigations
• PT-to investigate efficiency of the extrinsic pathway of coagulation
• APTT-to investigate efficiency of the intrinsic pathway of coagulation
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Investigations of a suspected vW disease
• Bleeding tendency due to absence of vWF,most common inherited bleeding disorder
• Easy bruising and mucocutaneous bleeding
• It is an autosomal dominant deficiency
• Should be considered in individuals with a history of bleeding,who show a prolonged BT and APTT
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• Types of vW disease type -1 type-2 type-3• type 1 and 3 are quantitative defects• Type 2 is qualitative defect
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vW factor• Huge molecule• Exists as multimers of large,medium and small
sizes• Associated with factor VIII• Rises with stress
• Blood type O have lowest amount• Blodd type AB have highest amount
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Investigations• Measurement of conc. of all factor VIII activities i.e
VIII C and Vw antigen
• vWF multimers-it shows the structure of the vWF
• PFA 100-closure time increased
• ELISA for vW antigen can also be done
• Further invetigation-immunoelectrophoresis of vW antigen
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Investigations of a suspected haemophilia disease
• X-linked recessive inheritance • Mainly affects males• Two types of haemophilia -haemophilia A-resulting due to
deficiency of factor VIII C -haemophilia B-resulting due to
deficiency of factor IX
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Investigations
• Platelet count-normal• BT –normal• CT-normal• PT-normal• APTT-prolonged• Factor VIII and IX assays-decreased
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