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3rd Year - Haematology > 6. Bleeding Disorders > Flashcards

6. Bleeding Disorders Flashcards

1
Q

What is the Normal Haemostatic Response to Cut?

A 
Primary - Platelet Plug Formation:
1. Platelets
2. Von Willebrand Factor
3. Vessel Wall
Secondary - Fibrin Plug Formation
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2
Q

What is Haemorrhagic Diathesis?

A

Any Quantitative / Qualitative Abnormality / Inhibiton of Function of:

  1. Platelets
  2. Von Willebrand Factor
  3. Coagulation Factors
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3
Q

What are the Important Factors to Cover in a Bleeding History?

A
  1. Has the Patient Actually got a Bleeding Disorder?
  2. How Severe is the Disorder?
  3. Pattern of Bleeding?
  4. Congenital / Acquired?
  5. Mode of Inheritance
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4
Q

What will appear in a History of Bleeding which will indicate a Bleeding Disorder?

A
  1. Bruising
  2. Epistaxis
  3. Post-Surgical Bleeding
  4. Menorrhagia
  5. Post-Partum Haemorrhage
  6. Post-Trauma
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5
Q

What are the Different Patterns of Bleeding?

A
  1. Platelet Type

2. Coagulation Factor

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6
Q

What are the Platelet Pattern Types of Bleeding?

A
  1. Mucosal
  2. Epistaxis
  3. Purpura
  4. Menorrhagia
  5. G.I.
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7
Q

What are the Coagulation Factor Pattern Types of Bleeding?

A
  1. Articular
  2. Muscle Haematoma
  3. CNS
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8
Q

How is a Bleeding Disorder distinguished between Congenital and Acquired?

A
  1. Previous Episodes
  2. Age at First Event
  3. Previous Surgical Challenges
  4. Associated History
    Note - Hereditary Disorder = Family Members with Similar History / Sex
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9
Q

What is the Aetiology of Haemophilia A and B?

A
  1. X-Linked - Identical Phenotypes
  2. Severity of Bleeding Depends on Residual Coagulation Factor Activity - <1% Severe / 1-5% Moderate / 5-30% Mild
  3. Haemophilia A = Reduced Factor VIII
  4. Haemophilia B = Reduced Factor IX
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10
Q

What are the Clinical Features of Haemophilia?

A
  1. Haemarthrosis
  2. Muscle Haematoma
  3. CNS Bleeding
  4. Retroperitoneal Bleeding
  5. Post-Surgical Bleeding
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11
Q

What are the Clinical Complications of Haemophilia?

A
  1. Synovitis
  2. Chronic Haemophilic Arthropathy
  3. Neurovascular Compression (Compartment Syndrome)
  4. Other Sequelae of Bleeding (Stroke)
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12
Q

How is a Diagnosis of Haemophilia made?

A
  1. Clinical
  2. Prolonged Activated Partial Thromboplastin Time (APTT)
  3. Normal Prothrombin Time (PT) - Different Part (Extrinsic Arm) of the Coagulation Cascade
  4. Normal Bleeding Time (BT) - Measures Only the Platelet Response to Vascular Injury
  5. Reduced Factor VIII
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13
Q

What is the Treatment of Bleeding Diathesis in Haemophilia?

A

Prophylaxis:
1. Coagulation Factor Replacement (A = FVIII / B = FIX)
2. Tranexamic Acid
3. DDAVP Tablets
Note - Now almost entirely Recombinant Products

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14
Q

What are the Treatments of Haemophilia?

A
  1. Splints
  2. Physiotherapy
  3. Analgesia
  4. Synovectomy
  5. Joint Replacement
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15
Q

What are the Complications of Haemophilia Treatment?

A
  1. Viral Infection - HIV / HBV / HCV
  2. Inhibitors - Anti-Factor VIII Antibody
  3. DDAVP = M.I. / Hyponatraemia (Babies)
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16
Q

What are the Features of Von Willebrand’s Disease?

A
  1. Common
  2. Variable Severity
  3. Autosomal
  4. Platelet Type Bleeding (Mucosal)
  5. Quantitative and Qualitative Abnormalities of Von Willebrand Factor
17
Q

What are the Different Types of Von Willebrand Disease?

A

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

18
Q

What is the Treatment of Von Willebrand Disease?

A
  1. Von Willebrand Factor Concentrates / DDAVP
  2. Tranexamic Acid
  3. Topical Applications
  4. Oral Contraceptive Pill
19
Q

What are the Different Acquired Bleeding Disorders?

A
  1. Thrombocytopenia
  2. Liver Failure
  3. Renal Failure
  4. Disseminated Intravascular Coagulation
  5. Drugs - Warfarin / Heparin / Aspirin / Clopidogrel
20
Q

What are the Causes of Thrombocytopenia?

A
  1. Decreased Production:
  2. a) Marrow Failure
  3. b) Aplasia
  4. c) Infiltration
  5. Increased Consumption:
  6. a) Immune Idiopathic Thrombocytopenic Purpura (ITP)
  7. b) Non-Immune Disseminated Intravascular Coagulation
  8. c) Hypersplenism
21
Q

What are the Clinical Features of Thrombocytopenia?

A
  1. Petechia
  2. Ecchymosis
  3. Mucosal Bleeding
  4. CNS Bleeding (Rare)
22
Q

What is Immune Idiopathic Thrombocytopenic Purpura (ITP) associated with?

A
  1. Infection - EBV / HIV
  2. Collagenosis
  3. Lymphoma
  4. Drug Induced
23
Q

What are the Treatments for Immune Idiopathic Thrombocytopenic Purpura (ITP)?

A
  1. Steroids
  2. I.V. IgG
  3. Splenectomy
  4. Thrombopoietin Analogues
24
Q

What are the Features of Liver Failure?

A
  1. Altered Factor I / II / V / VII / VIII / IX / X / XI
  2. Prolonged PT
  3. Prolonged APTT
  4. Reduced Fibrinogen
  5. Cholestasis - Vitamin K Dependent Factor Deficiency
  6. Treated with Replaced FFP / Vitamina K / Factor II / Factor VII / Factor IX / Factor X
25
Q

What are the Features of Haemorrhagic Disease of the Newborn?

A
  1. Immature Coagulation System
  2. Vitamin K Deficient Diet
  3. Fatal and Incapacitating Haemorrhage
  4. Completely Preventable by Administration of Vitamin K at Birth

3rd Year - Haematology (10 decks)

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