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BLEEDING DISORDERSSS.pptx
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- 2. OUTLINE • Introduction • Identifyingclotting factors • Physiology of Blood Clotting • ◦ Hemostasis • ◦ Bleeding disorders • -Vascular disorders • -Coagulation disorders • -Platelet disorders
- 3. INTRODUCTION • A groupof disorders characterized by defective haemostasis with abnormal bleeding. • These causes are broadly divided into 4 groups : • 1) Due to vascular abnormality. • 2) Due to platelet abnormality. • 3) Disorder of coagulation factor. • 4) Combination of all these as in disseminated intravascular coagulation.
- 5. HAEMOSTASIS • Primary Haemostasis ◦Blood vessel contraction ◦ Platelet Plug Formation • Secondary Haemostasis ◦ Activation of Clotting Cascade ◦ Deposition & Stabilization of Fibrin • Tertiary Haemostasis ◦ Dissolution of Fibrin Clot ◦ Dependent on Plasminogen Activation
- 6. CLOTTING FACTORS • FactorI : Fibrinogen • Factor II : Prothrombin • Factor III : Thromboplastin • Factor IV : Calcium • Factor V : Labile factor • Factor VII : Stable factor • Factor VIII: Antihemophilic factor • Factor IX: Christmas factor • Factor X: Stuart Power factor • Factor XI: Plasma thromboplastin antecedent • Factor XII: Hegman factor • Factor XIII : Fibrin stabilising factor
- 9. • BLEEDING TIME:Provides assessment of platelet count and function Normal value: 2-8 minutes. • PROTHROMBIN TIME: Measures Effectiveness of the Extrinsic Pathway Mnemonic - PET NORMAL VALUE: 10-15 SECS. • PARTIAL THROMBOPLASTIN TIME: Measures effectiveness of the Intrinsic Pathway Mnemonic - PITT NORMAL VALUE: 25-40 SECS
- 10. • THROMBIN TIME:Time for thrombin to convert fibrinogen to fibrin A measure of fibrinolytic pathway • Normal value : 9-13 secs
- 12. DISORDERS OF HAEMOSTASIS •Disorders of Blood vessels Scurvy, Henoch-Schonlein syndrome. • Disorders of Coagulation Extrinsic, intrinsic, combined. • Disorders of Platelets Thrombocytopenia ITP, TTP, HUS, DIC. Aspirin therapy, Thrombasthenia, • Other disorders DIC.
- 13. DISORDERS OF COAGULATION HaemophiliaA SYMPTOMS: • Bruising • Spontaneous bleeding • Bleeding into joints and associated • pain and swelling • Gastrointestinal tract and urinary tract hemorrhage • Prolonged bleeding from cuts, tooth extraction, and surgery • People whose clotting activity is 5percent of normal may have only mild hemophilia.
- 16. TREATMENT: • Infusion ofcryoprecipitate or desmopressin Acetate (ddavp) • Desmopressin injection or stimate nasal Spray. • Cryoprecipitate • Avoid certain drugs that can aggravate bleeding problems: • Aspirin, Heparin, Warfarin, Certain analgesics such as nonsteroidal anti-inflammatory drugs
- 17. HAEMOPHILIA B • Christmasdisease- deficiency in clotting factor IX. • Symptoms include: • Nose bleeds , Bruising • Spontaneous bleeding • Bleeding into joints and associated pain and swelling • Gastrointestinal tract and Urinary tract hemorrhage • Prolonged bleeding from cuts, tooth extraction, surgery, • following circumcision • People whose clotting activity is 5% of normal may have only mild hemophilia.
- 19. COMPLICATIONS • Chronic jointdeformities, • Intracerebral hemorrhage may also occur.
- 20. Von Willebrand Disease: •hereditary deficiency or abnormality of the von Willebrand factor in the blood, a protein that affects platelet function • .
- 21. Von Willebrand Disease Classifications: •Type I: Most common and mildest form of von Willebrand disease. Levels of von Willebrand factor are lower than normal, reduced levels of factor VIII. • Type II: • Von Willebrand factor itself has an abnormality.
- 22. V • Type III: •Severe von Willebrand disease. Total absence of von Willebrand factor, and factor VIII levels are often less than 10%. • Inheritance Pattern • occurs in men and women equally. • Types I and II are usually inherited in what is known as a "dominant“ pattern. • Type III von Willebrand disease, however, is usually inherited in a "recessive" pattern.
- 24. VITAMIN K DEFICIENCY •Source of vitamin K: Green vegetables Synthesized by intestinal flora • Required for synthesis Factors II, VII, IX ,X Protein C and S • Causes of deficiency: Malnutrition Biliary obstruction Malabsorption Antibiotic therapy • Treatment Vitamin K Fresh frozen plasma
- 25. CLASSIFICATION OF PLATELET DISORDERS •Quantitative disorders Decreased production Increased destruction. • Qualitative disorders • ◦ Inherited disorders (rare) • ◦ Acquired disorders Medications Chronic renal failure
- 26. THROMBOCYTOPENIA • Definition: • Thrombocytopeniais any disorder in which the platelet count is below 1OO,000/CC OF BLOOD. • THROMBOCYTOPENIA • Immune-mediated a. Idioapthic b. Drug-induced c. Collagen vascular disease d. Lympho proliferative disease e. Sarcoidosis • Non-immune mediated a) DIC b) Microangiopathic haemolytic anaemia
- 27. THROMBOCYTOPENIA • Thrombocytopenia isoften divided into three major causes of low platelets: • 1) Low production of platelets in the bone marrow • 2) Increased breakdown of platelets in the bloodstream (called • intravascular) • 3) Increased breakdown of platelets in the spleen or liver (called • extravascular • 1)Disorders that involve low production in the bone marrow include: • -Aplastic anemia • -Cancer in the bone marrow • -Infections in the bone marrow (rare) • -Drugs (very rare)
- 28. THROMBOCYTOPENIA • 2)Disorders thatinvolve the breakdown of platelets include: • Immune thrombocytopenic purpura (ITP) • Drug-induced immune thrombocytopenia • Drug-induced non immune thrombocytopenia • Thrombotic thrombocytopenic purpura • Disseminated intravascular coagulation (DIC) • Hypersplenism (an enlarged spleen)
- 29. THROMBOCYTOPENIA Symptoms: • Bruising • -Nosebleeds or bleeding in the mouth • ` -Rash (pinpoint red spots) • -Other symptoms may be present as well, depending on the • cause of the condition. • Mild thrombocytopenia can occur without symptoms.
- 30. THROMBOCYTOPENIA INVESTIGATIONS • FBC showslow platelets • -Bone marrow aspiration or Biopsy may be normal or may show • low megakaryocytes (platelet precursors) or an infiltrating disease. • -PTT clotting study is normal • -PT clotting study is normal • -Platelet associated antibodies may be present
- 31. THROMBOCYTOPENIA • Treatment • Dependson the cause of the condition. In some cases, transfusion of platelets may be required to stop or prevent bleeding. • Complications: • - Hemorrhage • -Gastro intestinal bleeding • -Intracranial haemorrhage
- 32. FRESH FROZEN PLASMA •Content - plasma (decreased factor V and VIII) • Indications Multiple coagulation deficiencies (liver disease, trauma) DIC Warfarin reversal Coagulation deficiency (factor XI or VII) • Dose (225 ml/unit) • 10-15 ml/kg • Viral screened product • ABO compatible
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- 34. CRYOPRECIPITATE • Prepared fromFFP • Content - fibrinogen, von Willebrand factor, factor VIII, factor XIII and fibronectin Indications • Fibrinogen deficiency • Uraemia • von Willebrand disease Dose (1 unit = 1 bag) • ◦ 1-2 units/10 kg body weight
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- 36. TREATMENT APPROACHES TOTHE BLEEDING PATIENT • Red blood cells • Platelet transfusions • Fresh frozen plasma • Cryoprecipitate • DDAVP • Recombinant Human factor VIIa
- 37. RBC TRANSFUSION: ADVERSE REACTIONS •Non-immunologic reactions • Congestive heart failure • Volume overload • Fever • Bacterial contamination • Hypocalcemia • Massive transfusion
- 38. PLATELET TRANSFUSION • Source Plateletconcentrate (Random donor) Pheresis platelets (Single donor) • Target level Bone marrow suppressed patient (>10- 20,000/μl) Bleeding (>50,000/μl)
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