Bleeding Disorders Flashcards
What is arterial thrombosis
When a blod clot forms in the arterial system
Risk factors for arterial thrombosis
Increasing age Smoking Sedentary lifestyle Hypertension DM Obesity Hypercholesterolemia
Describe the clot in arterial thrombosis
Platelets and fibrin
White clot
Pathology of arterial thrombosis
Results in infarction and ischaemia
Decrease in O2 supply
Example of arterial thrombosis
Coronary thrombosis disease:
MI
Angina
Cerebrovascular disease:
Stroke
TIA
Peripheral Disease:
Acute limb ischaemia
Chronic limb ischaemia
Acute presentation Rx for arterial thrombosis
Thrombolysis
Anti-platelet/anti-coagulant therapy
Interventional techniques e.g PCI
Primary Rx for arterial thrombosis
Lifestyle modification
Smoking cessation
Exercise
Diet ect..
What is a venous thrombosis
Thrombus arising in the venous system
Risk factors for venous thrombosis
Increasing age Pregnancy Hormonal therapy (COCP/HRT) Depoprovera (injection) higher risk than the other progesterone only methods) Tissue trauma Immobility Surgery Obesity Systemic disease FH (mainly related to 1st degree relatives
Describe the clot in venous thrombosis
Fibrin and red cells
Which aspects of Virchow’s triad tend to be most affected in venous disease
Blood flow
Blood constituents
What are the 2 main clinical manifestations of venous thrombosis disease
Limb DVT
PE
Rarer clinical manifestations of venous thrombosis disease
Visceral venous thrombosis
Intracranial venous thrombosis
Superficial thrombophlebitis
Which Pre-test scores can be used in venous thrombosis disease
Wells Score
Geneva Score
If probability score is low what is the next Ix
D-dimer
If probability score is high what is the next Ix
D-dimer skipped
Go straight to imaging
What is D-dimer a breakdown product of?
Fibrinolysis
Imaging Ix for venous thrombosis
Doppler USS
V/Q scan
CT Pulmonary Angiogram
Drug Rx for venous thrombosis
Anti-coagulants
LMWH
Warfarin
DOACs
Example of case where thrombolysis would be used
Massive PE
What is heritable thrombophilia
Inherited predisposition to venous thrombosis
Most common type of heritable thrombophilia
Factor V Leiden
Prothrombin G20210A
Rare types of heritable thrombophilia
Anti-thrombin deficiency
Protein C deficiency
Protein S deficiency
Common type of Acquired haemophilia
Anti-phospholipid syndrome
What conditions are most associated with heritable thrombophilia
DVT
PE
Ix for thrombophilia
FBC
Blood film
Clotting: PT Thrombin time APTT Fibrinogen
Rx for thrombophilia
No specific Rx
Who is thrombophilia prophylaxis not required for
Absence of VTE
But advise about risk with COCP or HRT
Who is prophylaxis required for in thrombophilia
Long term anticoagulation in those with recurrent episodes with no other risk factors
Who should be tested for thrombophilia
Arterial thrombus or MI <50yrs Unprovoked VTE <40yrs with no risk factors VTE with oral contraceptive/pregnancy Unusual site thrombosis e.g mesenteric Recurrent fetal loss Neonatal thrombosis
Pathology of prothrombin G20210A
Produce too much prothrombin
Increased tendency to clot
Increased risk of thrombosis
Pathology of anti-thrombin defiency
Deficit in anti-thrombin
Anti-thrombin is a naturally occurring anti-coagulant
Pathology of Protein C deficiency
Protein C is naturally occurring anti-coagulant
=> deficiency leads to increased risk of clots
What is protein S
Naturally occurring anti-coagulant
What does DIC stand for
Diffuse systemic coagulation activation
When can DIC typically occur
Septicaemia
Malignancy
Eclampsia
Severe burns
Pathology of DIC
Platelet and/or fibrin
Results in diffuse ischaemia affecting extremities
Rx for DIC
Mainly directed towards underlying conditions
Pathology of Factor V Leiden Syndrome
Hypercoagulable state
Prone to thrombosis
Mutated factor 5 is resistant to action of Protein C
Protein C is a natural anti-coagulant
Increased risk of developing DVT (PE) at some point
Rx for Factor V Leiden Syndrome
No cure
Prevention of thrombotic complications using medication
Miscarriage risk in Factor V Leiden Syndrome
Women with Factor V Leiden 2-3x risk of miscarriage
What is deficient in Haemophilia A
Factor 8
What is deficient in Haemophilia B
Factor 9
Inheritance pattern Haemophili
X-linked
What does the severity of bleeding in haemophilia depend on
Residual coagulation factor activity
What is common in haemophilia with factor level <1%
severe
Common to have spontaneous bleeding
Clinical features of haemophilia
Haemoarthrosis Muscle haematoma Post surgical bleeding Typically M 6mnth to 1yr
Which gender is affected by haemophilia
M
Ix for haemophilia
Clinical
Prolonged APTT
Normal PT
Normal BT
Reduced
Factor 8 or 9 depending
Rx for haemophilia
F VIII
F IX
Injections
Prevention Rx for haemophilia
Aim to prevent spontaneous bleeding Splints Physio. Analgesia Synovectomy Joint replacement
Complications of bleeding into muscles
Increased pressure
can cause:
Nerve palsies
Compartment syndrome
What blood cell does Von Willebrand Disease affect
Platelets
What is the commonest inheritable bleeding disorder
Von Willebrand Disease
Inheritance pattern of Von-Willebrand disease
Autosomal dominant
What pattern of bleeding is seen in Von-Willebrand disease
Mucosal pattern of bleeding
What are the 3 types of Von-Willebrand Disease
Type I, II, III
Describe Type I Von Willebrand disease
Mildest from
Quantitative defect
Don’t make enough
Describe Type II Von Willebrand Disease
Qualitative deficiency
Quality of the protein is not good enough
Doesn’t work properly
Describe Type III Von Willebrand disease
Severe (complete deficiency)
Most severe
Rarest
Clinical features of Von willebrand isease
Mucosal pattern of bleeding Easily bruising Large bruises Frequent/prolonged epistaxis Bleeding gums Heavy/prolonged bleeding from cuts Menorrhagia PPH (obs)
Rx for Von Willebrand disease
wVF concenrate DDAVP Tranexamic acid Topical applicaitons OCP
What is thrombocytopenia
Low platelet count
what are the causes of thrombocytopenia
Decreased production
Increased consumption
Decreased production causes of thrombocytopenia
Marrow failure
Aplasia
Infiltration
Increased consumption causes of thrombocytopenia
Immune ITP
Non-immune DIC
Hypersplenism
Clinical features of thrombocytopenia
Petechia
Ecchymosis
Mucosal pattern bleeding
Rare CNS bleeding
What is Immune ITP
Defined as low platelet count with normal bone marrow
And absence of other causes of thrombocytopenia
What causes immune ITP
Anti-platelet antibodies
Autoimmune
Associations of Immune ITP
Infection esp. EBV, HIV
Colagenosis
Lymphoma
Drug induced (quinine esp.)
2 distinct patterns of Immune ITP
Actue
Chronic
Typical picture of acute immune ITP
Children
Following acute infection
Self-limiting purpura
Typical picture of chronic immune ITP
Adults Mainly women Fluctuating course Bleeding Epistaxis Menorrhagia
Clinical picture of immune ITP
Increased tendency to bleed Petechiae Purpura Menorrhagia Epistaxis Bleeding from gums
Ix for Immune ITP
Isolated thrombocytopenia
Only abnormality = > easy to Dx
Diagnosis of exclusion
Bone marrow?
Very rarely need to do tis to Dx
Rx for Immune ITP
Steroids:
Dexamethasone
Prednisolone
IgG transfusion
Thrombopoietin analogous:
Romiplostim
Oral thrombopoietin receptor agonist:
Eltrombopag
What is Immune ITP a Dx of
Dx of exclusion
