Bleeding Disorders: Classification and Diagnosis

These slides briefly tell about the bleeding disorders, their classification and diagnosis .

1. Presented by: Rajat Hegde Bleeding disorders: classification and diagnosis

2. contents Introduction Hemostasis Coagulation cascade Classification Diagnosis Laboratory tests Conclusion References

3. Introduction Bleeding disorders are rare disorders affecting the way the body controls bleeding. Excessive bleeding can result from: 1. Increased fragility of vessels 2. Platelet deficiency or dysfunction 3. Derangement of coagulation The normal hemostatic response involves the blood vessel wall, the platelets and the clotting cascade. Various laboratory tests are used in evaluation of a bleeding diathesis.

4. Blood vessel injury triggers the following sequence: Vessel constriction. Circulating platelets adhere to vessel. A series of enzymatic reactions involving coagulation proteins, produces fibrin to form a stable hemostatic plug.

5. hemostasis Hemostasis is the mechanism that leads to cessation of bleeding from a blood vessel. Primary Hemostasis: Platelet plug formation at sites of injury. Secondary Hemostasis: Plasma coagulation system reaction resulting in fibrin formation.

6. Coagulation cascade

7. classification I) Coagulation Factor Deficiency: Congenital: Hemophilia A and B v on Willebrand Disease Other factor deficiencies II) Fibrinolytic Defects: Streptokinase therapy DIC Acquired Liver disease Vitamin K deficiency DIC

8. III) Vascular Disorders: Scurvy Purpura Hereditary hemorrhagic telangiectasia Cushing syndrome Ehlers Danlos syndrome

9. IV) Platelet Disorders: 1] Quantitative Disorders (Thrombocytopenia): 2] Qualitative Disorders : Congenital: Glanzmann thrombasthenia von Willebrand disease Acquired: Liver disease Drug-induced Alcoholism Immune Mediated: Idiopathic Collagen vascular disease Sarcoidosis Non-Immune Mediated: Microangiopathic hemolytic anemia Leukemia Myelofibrosis

10. Diagnosis of bleeding disorders Terminologies: Petechiae : small, pinpoint bleeding spot, measures 1-2 mm. Purpura: red, non-blanching maculo-paular lesions caused by intradermal capillary bleeding. Measures ≥ 3mm. Ecchymosis: A discoloration of skin resulting from bleeding underneath, typically caused by bruising. Measures ≥ 1cm. Hematoma: A pool of partially clotted blood in an organ, tissue or body space, usually caused by a broken blood vessel.

11. It’s important to have relevant clinical information before interpreting results. From the history, it’s important to differentiate hereditary disorders from acquired. Hereditary disorders generally start from an early age or recurrent in nature and may have family history. Acquired disorders on the other hand occur at any age and have an underlying predisposing cause. Depending on type of bleeding, it’s important to differentiate between platelet and factor bleeds.

12. History of medication should include replacement therapy, use of anticoagulants like heparin besides intake of analgesics and antib iotics etc . In general, symptoms of: 1. Platelet bleeds: petechial hemorrhage, mucosal bleed. 2. Coagulation factor disorders: deep abdominal bleeds, muscle bleeds, hemostasis, ecchymotic patches.

13. Laboratory tests Bleeding time Clotting time Platelet count Activated partial thromboplastin time ( aPTT ) Prothrombin time (PT) Thrombin time (TT) D-dimer Bleeding Time: It’s the time from puncture of skin and stoppage of blood oozing out. Normal Range: 2 – 5 mins Prolonged in platelet disorders: thrombocytopenia, thrombocytopenic purpura.

14. Clotting Time: It’s the time required for blood to form a clot. Normal Range: 4 - 10 mins Prolonged in: Hemophilias , vitamin K deficiency. a PTT (Activated Partial Thromboplastin Time): It’s performed by adding a surface activator (like kaolin/ ellagic acid), phospholipid and calcium to patient’s plasma. Normal Range: 26 – 37 sec Prolonged aPTT : Hemophilia A and B, von Willebrand disease, DIC, heparin therapy.

15. Prothrombin Time: It’s measured by adding tissue factor (thromboplastin) and calcium to the patient’s plasma. Normal Range: 12 – 16 sec Prolonged in: liver diseases, DIC, vitamin K deficiency, D-dimer: It’s a fibrin degradation product, a small protein fragment present in the blood after a blood clot is degraded by fibrinolysis. It contains 2 cross-linked D fragments of the fibrinogen protein. D-dimer concentration helps to diagnose thrombosis. While negative result practically rules out thrombosis, positive result can indicate thrombosis but does not rule out other potential causes.

16. conclusion The medical and family history is very important in evaluating patients with bleeding disorders. Dental extractions are very common major stresses of hemostatic mechanism, and a prior history is very important. So, a thorough understanding and knowledge about bleeding disorders is very much needed for dental professionals to minimise the complications of many treatment procedures.

17. REFERENCES Robbins and Cottran Pathologic Basis of Disease. Davidson’s Essentials of Medicine.

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