Bleeding Disorders Flashcards
What are the two main categories of bleeding disorders?
Congenital and Acquired
Congenital disorders are present from birth, while acquired disorders develop later in life.
What are the normal haemostatic mechanisms involved in bleeding control?
Vessel Wall, Platelets, von Willebrand Factor, Coagulation Factors
These mechanisms work together to prevent excessive bleeding.
What is the primary phase of the haemostatic response?
Platelet Plug Formation
This phase involves the aggregation of platelets at the site of injury.
What is the secondary phase of the haemostatic response?
Fibrin Plug Formation
This phase involves the formation of a stable fibrin clot.
What does haemorrhagic diathesis refer to?
Any quantitative or qualitative abnormality inhibiting function of platelets, vWF, or coagulation factors
It indicates a predisposition to bleeding.
What are the key points to assess 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
A thorough bleeding history is critical for diagnosis.
What are common patterns of bleeding associated with platelet disorders?
- Mucosal
- Epistaxis
- Purpura
- Menorrhagia
- GI
These symptoms often indicate issues with platelet function.
What are common patterns of bleeding associated with coagulation factor disorders?
- Articular
- Muscle Haematoma
- CNS
- Rectus Sheath Haematoma
- Intracranial Haemorrhage
These symptoms suggest problems with coagulation factors.
What is the mode of inheritance for Haemophilia A and B?
X-linked
Both types of haemophilia are inherited through the X chromosome.
What are the severity classifications for haemophilia based on residual coagulation factor activity?
- <1% Severe
- 1-5% Moderate
- 5-30% Mild
The classification helps determine treatment strategies.
What are the clinical features of haemophilia?
- Haemarthrosis
- Muscle haematoma
- CNS bleeding
- Retroperitoneal bleeding
- Post surgical bleeding
These features are indicative of bleeding diathesis.
What is the typical diagnostic procedure for haemophilia?
- Clinical assessment
- Family history
- Pattern of bleeding
- Isolated prolonged APTT
- Normal PT
- Reduced FVIII or FIX
- Genetic analysis
These steps ensure accurate diagnosis.
What are the treatment options for severe haemophilia?
- Prophylaxis with recombinant factor concentrates
- Emicizumab
- On demand factor for bleeding episodes
- Extended half-life factor concentrate
Treatment strategies vary based on severity and patient needs.
What is Emicizumab?
A recombinant, humanised, bispecific monoclonal antibody that bridges activated factor IX and factor X
It mimics the function of missing activated factor VIII.
What is von Willebrand Disease?
A common bleeding disorder with variable severity, characterized by quantitative and qualitative abnormalities of vWF
It is the most prevalent inherited bleeding disorder.
What are the types of von Willebrand Disease?
- Type 1 - Quantitative deficiency
- Type 2 (A, B, M, N) - Qualitative deficiency
- Type 3 - Severe (complete) deficiency
Each type has different implications for treatment.
What are common acquired bleeding disorders?
- Thrombocytopenia
- Liver failure
- Renal failure
- DIC
- Drugs (e.g., Warfarin, Heparin)
These conditions can lead to significant bleeding risks.
What are the clinical features of thrombocytopenia?
- Petechia
- Ecchymosis
- Mucosal bleeding
- Rare CNS bleeding
Thrombocytopenia often results in easy bruising and bleeding.
What is the management approach for immune thrombocytopenic purpura (ITP)?
- Acute - Steroids, IVIg
- Long-term - Thrombopoietin analogues, Immunosuppression
- Splenectomy - Rare these days
Management strategies depend on whether the condition is acute or chronic.
What is the primary prevention for Haemorrhagic Disease of the Newborn?
Administration of vitamin K at birth
This condition is preventable and can lead to severe bleeding if not treated.
True or False: Bleeding disorders can result from disruption at any stage of haemostasis.
True
Understanding the stages of haemostasis is crucial for diagnosing and managing bleeding disorders.
Extended half life factor concentrate
. In adults and adolescents (≥12 years)
EHL-FVIII products have an average increase in half-life of about 1.5 times compared to the standard FVIII concentrates
EHL-FIX products have a 3–5 fold increase in half-life compared to standard FIX concentrates
Emicizumab
Recombinant, humanised, bispecific monoclonal antibody
Bridges activated factor IX and factor X, mimicking the function of missing activated factor VIII
Prophylaxis
Subcutaneous
Can be used in patients with inhibitors
Gene therapy
Adeno-associated virus (AAV) vector that carries a copy of the factor VIII/ IX gene
Hemgenix (Factor IX)
Mild haemophilia
Treatment
On demand
Bleeding episodes and peri-operatively
Recombinant factor concentrate
DDAVP (Haemophilia A)
Tranexamic Acid
Severe haemophilia treatment
Prophylaxis
Recombinant factor concentrates
EHL or SHL
Emicizumab for haemophilia A
On demand factor for bleeding episodes
