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What is the main cause of mucosal bleeding?
What is the main cause of mucosal bleeding?
What is the purpose of the history and examination in bleeding disorders?
What is the purpose of the history and examination in bleeding disorders?
What is the result of fibrinolysis?
What is the result of fibrinolysis?
What is the definition of pathological bleeding?
What is the definition of pathological bleeding?
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What is the result of limiting clot formation?
What is the result of limiting clot formation?
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What is the early haemostatic response?
What is the early haemostatic response?
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What is the result of stage 1 of normal haemostasis?
What is the result of stage 1 of normal haemostasis?
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What is the common feature of bleeding into joints or muscles?
What is the common feature of bleeding into joints or muscles?
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What is the name of the disease caused by Factor VIII deficiency?
What is the name of the disease caused by Factor VIII deficiency?
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Where is the factor VIII gene located?
Where is the factor VIII gene located?
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What is the primary function of von Willebrand factor (vWF)?
What is the primary function of von Willebrand factor (vWF)?
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What is the half-life of factor VIII?
What is the half-life of factor VIII?
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What is the treatment for bleeding in severe haemophilia A?
What is the treatment for bleeding in severe haemophilia A?
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What is the name of the disease caused by factor IX deficiency?
What is the name of the disease caused by factor IX deficiency?
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What is the treatment for mild Von Willebrand disease?
What is the treatment for mild Von Willebrand disease?
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What is the result of desmopressin treatment in Von Willebrand disease?
What is the result of desmopressin treatment in Von Willebrand disease?
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What may cause excessive fibrinolysis?
What may cause excessive fibrinolysis?
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What is not a reliable method for detecting all causes of pathological bleeding?
What is not a reliable method for detecting all causes of pathological bleeding?
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What indicates a likely defect in coagulation factors?
What indicates a likely defect in coagulation factors?
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What is characterized by minor bleeding into the dermis that is flat and non-blanching?
What is characterized by minor bleeding into the dermis that is flat and non-blanching?
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What is more likely to be due to thrombocytopenia, a platelet function disorder, or von Willebrand disease?
What is more likely to be due to thrombocytopenia, a platelet function disorder, or von Willebrand disease?
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What suggests a local structural abnormality rather than coagulopathic bleeding?
What suggests a local structural abnormality rather than coagulopathic bleeding?
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What is characterized by bleeding into deeper layers of the skin, initially appearing as dark red or purple, and later turning yellow?
What is characterized by bleeding into deeper layers of the skin, initially appearing as dark red or purple, and later turning yellow?
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What is indicated by telangiectasia of lips and tongue?
What is indicated by telangiectasia of lips and tongue?
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What is the purpose of a full examination in the context of bleeding disorders?
What is the purpose of a full examination in the context of bleeding disorders?
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What is the cause of congenital platelet function disorders?
What is the cause of congenital platelet function disorders?
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What is the most common cause of acquired platelet function disorders?
What is the most common cause of acquired platelet function disorders?
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What is the typical platelet count at which spontaneous bleeding occurs in ITP?
What is the typical platelet count at which spontaneous bleeding occurs in ITP?
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What is the mechanism of idiopathic thrombocytopenic purpura (ITP)?
What is the mechanism of idiopathic thrombocytopenic purpura (ITP)?
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What is the underlying cause of some cases of ITP?
What is the underlying cause of some cases of ITP?
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What is the characteristic of thrombocytopenia?
What is the characteristic of thrombocytopenia?
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What is the underlying cause of bleeding disorders related to vessel wall abnormalities?
What is the underlying cause of bleeding disorders related to vessel wall abnormalities?
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What is the mainstay of treatment for all forms of VTE?
What is the mainstay of treatment for all forms of VTE?
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What is the target INR for patients treated with warfarin?
What is the target INR for patients treated with warfarin?
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What is the minimum duration of treatment with LMWH?
What is the minimum duration of treatment with LMWH?
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What is the characteristic of disseminated intravascular coagulation?
What is the characteristic of disseminated intravascular coagulation?
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What is the primary aim of therapy in disseminated intravascular coagulation?
What is the primary aim of therapy in disseminated intravascular coagulation?
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What is the effect of systemic activation of coagulation in disseminated intravascular coagulation?
What is the effect of systemic activation of coagulation in disseminated intravascular coagulation?
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What is the common complication of illnesses that may lead to disseminated intravascular coagulation?
What is the common complication of illnesses that may lead to disseminated intravascular coagulation?
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What is the role of tranexamic acid in bleeding disorders?
What is the role of tranexamic acid in bleeding disorders?
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Study Notes
Bleeding Disorders
- Bleeding can be due to congenital or acquired abnormalities in the clotting system.
- History and examination help to clarify the severity and the underlying cause of the bleeding problem.
Patterns of Bleeding
- Mucosal bleeding:
- Reduced number or function of platelets (e.g. bone marrow failure or aspirin)
- Deficiency of von Willebrand factor (e.g. von Willebrand disease)
- Characterized by:
- Skin: petechiae, bruises
- Gum and mucous membrane bleeding
- Fundal haemorrhage
- Post-surgical bleeding
- Coagulation factor deficiency (e.g. haemophilia or warfarin/anticoagulant):
- Bleeding into joints (haemarthrosis) or muscles
- Bleeding into soft tissues
- Retroperitoneal haemorrhage
- Intracranial haemorrhage
- Post-surgical bleeding
Normal Haemostasis
- Stage 1: Pre-injury conditions encourage flow
- Stage 2: Early haemostatic response: platelets adhere; coagulation is activated
- Stage 3: Fibrin clot formation
- Stage 4: Limiting clot formation: natural anticoagulants reverse activation of coagulation factors
- Stage 5: Fibrinolysis: plasmin degrades fibrin to allow vessel recanalisation and tissue repair
Pathological Bleeding
- Normal bleeding is seen following surgery and trauma
- Pathological bleeding occurs when structurally abnormal vessels rupture or when a vessel is breached in the presence of a defect in haemostasis
- May be due to a deficiency or dysfunction of platelets, the coagulation factors, or von Willebrand factor, or occasionally to excessive fibrinolysis
Clinical Assessment
- A careful clinical evaluation is the key to diagnosis of bleeding disorders
- Consider the following:
- Site of bleeding
- Duration of history: congenital or acquired
- Precipitating causes: spontaneous indicates a more severe defect
- Surgery
- Family history
- Drugs history
- Site of bleeding
Causes of Bleeding Disorders
- Disorders of primary haemostasis:
- Vessel wall abnormalities (e.g. hereditary haemorrhagic telangiectasia, vasculitis, or scurvy)
- Platelet function disorders (e.g. Glanzmann's thrombasthenia, Bernard-Soulier syndrome)
- Thrombocytopenia:
- Occurs in many disease processes
- Can be immune-mediated or due to increased consumption or inhibition of function of coagulation factors
- Disorders of secondary haemostasis (coagulation):
- Haemophilia A (factor VIII deficiency)
- Haemophilia B (factor IX deficiency)
Haemophilia A
- Factor VIII deficiency resulting in haemophilia A affects 1/10,000 individuals
- Sex-linked disorder located on the X chromosome
- Factor VIII is primarily synthesised by the liver and endothelial cells and has a half-life of about 12 hours
Management of Haemophilia
- Bleeding in mild and moderate forms usually do not require specific treatment, just supportive treatment
- Severe forms require treatment with factor VIII or IX concentrates or cryoprecipitate
Von Willebrand Disease
- Common but usually mild bleeding disorder caused by a quantitative or qualitative deficiency of von Willebrand factor (vWF)
- vWF is synthesised by endothelial cells and megakaryocytes, and is involved in both platelet function and coagulation
Management of Von Willebrand Disease
- Many episodes of mild haemorrhage can be successfully treated by local means or with desmopressin, which raises the vWF level, resulting in a secondary increase in factor VIII
- Tranexamic acid may be useful in mucosal bleeding
- For more serious or persistent bleeds, haemostasis can be achieved with selected factor VIII concentrates, which contain considerable quantities of vWF in addition to factor VIII
Thrombotic Disorders
Venous Thromboembolic Disease
- Most common presentations: deep vein thrombosis (DVT) of the leg and/or pulmonary embolism
- Management:
- Anticoagulation with low-molecular-weight heparin (LMWH) followed by a coumarin anticoagulant, such as warfarin
- Alternatively, direct oral anticoagulants (DOACs) can be used immediately from diagnosis without the need for LMWH
Disseminated Intravascular Coagulation (DIC)
- Complicates a range of illnesses
- Characterised by systemic activation of the pathways involved in coagulation and its regulation
- May result in the generation of intravascular fibrin clots causing multi-organ failure, with simultaneous coagulation factor and platelet consumption, causing bleeding
Management of DIC
- Therapy is primarily aimed at the underlying cause
- Often require intensive care to deal with concomitant issues, such as acidosis, dehydration, renal failure, and hypoxia
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Description
This quiz covers bleeding disorders, including congenital and acquired abnormalities in the clotting system. It tests knowledge of the causes, symptoms, and diagnosis of bleeding problems.
