6. Bleeding Disorders Flashcards
What is the Normal Haemostatic Response to Cut?
Primary - Platelet Plug Formation: 1. Platelets 2. Von Willebrand Factor 3. Vessel Wall Secondary - Fibrin Plug Formation
What is Haemorrhagic Diathesis?
Any Quantitative / Qualitative Abnormality / Inhibiton of Function of:
- Platelets
- Von Willebrand Factor
- Coagulation Factors
What are the Important Factors to Cover in a Bleeding History?
- Has the Patient Actually got a Bleeding Disorder?
- How Severe is the Disorder?
- Pattern of Bleeding?
- Congenital / Acquired?
- Mode of Inheritance
What will appear in a History of Bleeding which will indicate a Bleeding Disorder?
- Bruising
- Epistaxis
- Post-Surgical Bleeding
- Menorrhagia
- Post-Partum Haemorrhage
- Post-Trauma
What are the Different Patterns of Bleeding?
- Platelet Type
2. Coagulation Factor
What are the Platelet Pattern Types of Bleeding?
- Mucosal
- Epistaxis
- Purpura
- Menorrhagia
- G.I.
What are the Coagulation Factor Pattern Types of Bleeding?
- Articular
- Muscle Haematoma
- CNS
How is a Bleeding Disorder distinguished between Congenital and Acquired?
- Previous Episodes
- Age at First Event
- Previous Surgical Challenges
- Associated History
Note - Hereditary Disorder = Family Members with Similar History / Sex
What is the Aetiology of Haemophilia A and B?
- X-Linked - Identical Phenotypes
- Severity of Bleeding Depends on Residual Coagulation Factor Activity - <1% Severe / 1-5% Moderate / 5-30% Mild
- Haemophilia A = Reduced Factor VIII
- Haemophilia B = Reduced Factor IX
What are the Clinical Features of Haemophilia?
- Haemarthrosis
- Muscle Haematoma
- CNS Bleeding
- Retroperitoneal Bleeding
- Post-Surgical Bleeding
What are the Clinical Complications of Haemophilia?
- Synovitis
- Chronic Haemophilic Arthropathy
- Neurovascular Compression (Compartment Syndrome)
- Other Sequelae of Bleeding (Stroke)
How is a Diagnosis of Haemophilia made?
- Clinical
- Prolonged Activated Partial Thromboplastin Time (APTT)
- Normal Prothrombin Time (PT) - Different Part (Extrinsic Arm) of the Coagulation Cascade
- Normal Bleeding Time (BT) - Measures Only the Platelet Response to Vascular Injury
- Reduced Factor VIII
What is the Treatment of Bleeding Diathesis in Haemophilia?
Prophylaxis:
1. Coagulation Factor Replacement (A = FVIII / B = FIX)
2. Tranexamic Acid
3. DDAVP Tablets
Note - Now almost entirely Recombinant Products
What are the Treatments of Haemophilia?
- Splints
- Physiotherapy
- Analgesia
- Synovectomy
- Joint Replacement
What are the Complications of Haemophilia Treatment?
- Viral Infection - HIV / HBV / HCV
- Inhibitors - Anti-Factor VIII Antibody
- DDAVP = M.I. / Hyponatraemia (Babies)
What are the Features of Von Willebrand’s Disease?
- Common
- Variable Severity
- Autosomal
- Platelet Type Bleeding (Mucosal)
- Quantitative and Qualitative Abnormalities of Von Willebrand Factor
What are the Different Types of Von Willebrand Disease?
Type 1 - Quantitative Deficiency
Type 2 - Qualitative Deficiency - determined by the site of Mutation in relation to the Von Willebrand Factor Function
Type 3 - Severe (Complete) Deficiency
What is the Treatment of Von Willebrand Disease?
- Von Willebrand Factor Concentrates / DDAVP
- Tranexamic Acid
- Topical Applications
- Oral Contraceptive Pill
What are the Different Acquired Bleeding Disorders?
- Thrombocytopenia
- Liver Failure
- Renal Failure
- Disseminated Intravascular Coagulation
- Drugs - Warfarin / Heparin / Aspirin / Clopidogrel
What are the Causes of Thrombocytopenia?
- Decreased Production:
- a) Marrow Failure
- b) Aplasia
- c) Infiltration
- Increased Consumption:
- a) Immune Idiopathic Thrombocytopenic Purpura (ITP)
- b) Non-Immune Disseminated Intravascular Coagulation
- c) Hypersplenism
What are the Clinical Features of Thrombocytopenia?
- Petechia
- Ecchymosis
- Mucosal Bleeding
- CNS Bleeding (Rare)
What is Immune Idiopathic Thrombocytopenic Purpura (ITP) associated with?
- Infection - EBV / HIV
- Collagenosis
- Lymphoma
- Drug Induced
What are the Treatments for Immune Idiopathic Thrombocytopenic Purpura (ITP)?
- Steroids
- I.V. IgG
- Splenectomy
- Thrombopoietin Analogues
What are the Features of Liver Failure?
- Altered Factor I / II / V / VII / VIII / IX / X / XI
- Prolonged PT
- Prolonged APTT
- Reduced Fibrinogen
- Cholestasis - Vitamin K Dependent Factor Deficiency
- Treated with Replaced FFP / Vitamina K / Factor II / Factor VII / Factor IX / Factor X
What are the Features of Haemorrhagic Disease of the Newborn?
- Immature Coagulation System
- Vitamin K Deficient Diet
- Fatal and Incapacitating Haemorrhage
- Completely Preventable by Administration of Vitamin K at Birth
