Bleeding Disorders Flashcards
What are involved in the Normal Haemostatic Mechanisms?
- Vessel Wall
- Platelets
- Von Willebrand Factor
- Coagulation Factors
What is involved in the Primary and Secondary Haemostatic Response?
Primary;
- Platelet Plug Formation
- Platelets, vWF, Wall
Secondary;
- Fibrin Plug Formation
What is important to assess when faces with Haemorrhagic Diathesis (Bleeding tendencies)
Any quantitative or qualitative abnormality
Inhibition of function
- Platelets
- vWF
- Coagulation factors
**Sometimes accompany hepatic failure. Impaired synthesis of clotting factors, reduced clearance of the products of the clotting process, and metabolic abnormalities affecting platelet function can affect normal clotting, individually or in combination.
REVIEW!
What should you establish in a Bleeding History?
- Has the patient actually got a bleeding disorder
- How severe is the disorder?
- Pattern of Bleeding
- Congenital or Acquired
- Mode of inheritance
What else would you ask in the History of Bleeding?
- Bruising (Mucosal, easy bleeding?)
- Epistaxis
Response to challenges
- Post-surgical bleeding
- Dental extractions
- Menorrhagia (Start when period started or later in life (different cause))
- Post-partum haemorrhage (Surgery or Dentist?)
- Post-trauma
What tool do we use to help diagnose Bleeding severity?
ISTH Bleeding Assessment Tool Score
Used to diagnose how significant the bleeding is, the severity and if it’s appropriate for the trauma caused
What should you ask about in the “Pattern Of Bleeding” History?
Platelet type:
Mucosal Bleeding? (More in line with primary, haemostatic, von willebands)
- Epistaxis
- Purpura
- Menorrhagia
- GI
Coagulation Factor:
Articular?
- Muscle Haematoma
- CNS
What is this? INSERT PICTURE
Bruising in petechial rash - low platelets. Need urgent platelet count, will be low
What is this? (INSERT PIC)
Knee haemarthrosis, knee haemophilia, bled into knee
What is this? (INSERT PIC)
Rectus Sheath Haematoma - Seen in warfarin and if over anti-coagulated or haemophilia
What is this? (INSERT PIC)
Intracranial Haemorrhage in Haemophilia - Very rare now with good care
What do you want to ask in “Congenital or Acquired?”
- Previous Episodes ?
- Age at first event
- Previous surgical challenges
- Associated History
If you think the disorder is Hereditary what should you establish?
- Family members with similar history
- Sex (or inheritance patterns)
What kind of inheritance is this? INSERT PIC
Autosomal dominant (von Willebrand’s)
What are the features of haemophilia A and B?
- X-Iinked
- Identical phenotypes
- 1 in 10,000 (A) and 1 in 60,000 (B)
- Severity of bleeding depends on the residual coagulation factor activity
- <1% Severe (Have to have very low factor level for this)
- 1-5% Moderate
- 5-30% Mild (Start to develop symptoms when 1 year old)
What Haemopholia is more common?
Haemophilia A 1 in 10,000 (B is 1 in 60,000)
What are the Clinical features of haemophilia A and B?
- Haemarthrosis
- Muscle haematoma
- CNS bleeding (disappearing nowadays)
- Retroperitoneal bleeding
- Post surgical bleeding
What is Haemophilia?
Coagulation factor defect so more bleeding
What is this? (INSERT PIC)
Severe Haemophilia and haemarthrosis
What is this? (INSERT PIC)
Chest wall haemoarthroma
What are the clinical complications of Haemophilia?
- Synovitis
- Chronic Haemophilic Arthropathy
- Neurovascular compression (compartment syndromes)
- Other sequelae of bleeding (Stroke)
What is this? INSERT PIC
Severe haemophilia arthropathy
How is Haemophilia diagnosed?
- Clinical (Suspicions from history)
*1st do routine coagulation screening - Isolated prolonged AP TT
- Normal PT
- Reduced F-Vlll or FIX (8 = Haemophilia A, 9 = Haemophilia B, intrinsic issues as these at clotting factors)
Genetic analysis (to confirm and offer to other family members)
Haemophilia Treatment
Splints
Physiotherapy
Analgesia
Synovectomy
Joint replacement
Haemophilia treatment (complications)
• Viral infection
• Inhibitors
D DAV P
HIV
HBV, HCV,
Others/ vCJD?
Anti FVIII Ab
Rare in FIX
Ml
Hyponatraemia(babies)
von Willebrand Disease
Common (1 in 200)
Variable severity
Autosomal
Platelet Type bleeding (mucosal)
Quantitative and qualitative abnormalities of vWF
Pic
Von Willebrand Disease
• Type 1 quantitative deficiency
• Type 2 (A,B,M,N) qualitative deficiency determined by the site of mutation in relation
to vWF function
• Type 3 severe (complete) deficiency
DI 02 0-03
04 Cl CZ
Von Willebrand Disease
• vWF concentrate or DDAVP
• Tranexamic Acid
• OCP etc
Acquired Bleeding Disorders
Thrombocytopenia
Liver failure
Renal failure
• DIC
Drugs Warfarin, Heparin, Aspirin, Clopidogrel, Rivaroxaban, Apixaban, Dabigatran,
Bivalirudin
Acquired clotting factors deficiencies - rare
Thrombocytopenia
• Decreased production
• Increased consumption
Marrow failure
Aplasia
Infiltration
Immune ITP
DIC
Hypersplenism
Thrombocytopenia Clinical
Petechia
Ecchymosis
Mucosal Bleeding
Rare CNS bleeding
Significant blood blisters - single platelets count
Petechial rashes - urgent platelet count if seen
• Adults vs Children
• Associations
Infection esp EBV,HIV
Lymphoma
Drug induced
• Blood picture = isolated thrombocytopenia
• Marrow ?
• Steroids, IV lgG, Thrombopoietin analogues (Eltrombopag and romiplostim),
Splenectomy - rare these days
Liver Failure
• Factor l, Il, V, Vil, X, Xl
Prolonged P T, AP TT Reduced Fibrinogen
• Cholestasis - Vit K dept factor deficiency
Factor Il, Vil, X
Procoagulant
Factors
Anticoagulant
Factors
Normal Hemostasis
Liver
isease
Fiøvte The cf end
Coagulation in liver disease
Haemorrhagic Disease of the Newborn
Immature Coagulation Systems
Vitamin K deficient diet (esp Breast)
Fatal and incapacitating haemorrhage
Completely preventable by administration of vitamin K at birth (I.M vs P.O)
