APPROACH TO A BLEEDING DISEASE

Presented by-DaminiB.Sc-M.L.T-3rd yearModerator-Dr.Anju

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

• 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

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.

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

• Severity of bleeding

bleeding after major surgery or trauma suggests only a mild bleeding disorder

• 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

Laboratory Approach

1. Demonstration of the defect2. Identification of the defect(s)3. Assessment of severity4. Consequential studies eg. carrier detection5. Monitoring of treatment

Screening Tests

1. Platelet count & morphology 2. Bleeding Time 3. Clotting Time 4. Prothrombin Time 5. Activated Partial Thromboplastin Time

6. Thrombin Time

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

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

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

PROSTAGLANDIN PATHWAYS• radio immuno assay• Studies with radioactive arachidonic acid• MDA assay using thiobarbituric acid

PLATELET COAGULANT ACTIVITY• Prothrombin consumption index

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

Investigations

• PT-to investigate efficiency of the extrinsic pathway of coagulation

• APTT-to investigate efficiency of the intrinsic pathway of coagulation

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

• Types of vW disease type -1 type-2 type-3• type 1 and 3 are quantitative defects• Type 2 is qualitative defect

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

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

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

Investigations

• Platelet count-normal• BT –normal• CT-normal• PT-normal• APTT-prolonged• Factor VIII and IX assays-decreased

THANK YOU