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Flashcards in Haematology Coagulation 2 Deck (76)
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1

Haemophilia: Description

Defect in Factors that affect clotting factors downstream

2

Haemophilia A

Defect in F8 gene causing reduced FVIII

3

Haemophilia B

Defect in F9 causing reduced FIX

4

Haemophilia: Inheritance

Sex Linked – FIND OUT MORE

5

Haemophilia: Clinical features of Haemophilia

Mild provoked bleeding if factor level >5%
Severe spontaneous bleeding if factor level <1%
• Soft tissue and joint bleedings (leads to synovitis) – chronic inflammatory changes
• Life-threatening CNS or GI bleeds
• Chronic arthropathy
• Treatment acquired HCV and HIV

6

Haemophilia: Treatment

Recombinant factor concentrate – personalised prophylaxis regimes to ensure factor levels never drop to ‘severe’ levels

→ Implant venous access device for easier infusions

7

Venous Thrombosis: VTE disease

Formation of fibrin-rich clots in low-pressure venous system
Includes DVT, PR, or thrombosis in axillary/ subclavian/portal. Mesenteric/cerebral veins

8

Venous Thrombosis: Thrombophilia

Increased propensity to VTE
Acquired + genetic risk factors

9

Venous Thrombosis: National burden of VTE

PE
DVT – 25K deaths per year in the UK

Elective Hip and Knee surgery % risk with no prophylaxis:
• 45% Hip
• 60% knee

10

Venous Thrombosis:DVT presentation common

Unilateral pain
Swelling
Tenderness
Discolouration

11

Venous Thrombosis: DVT presentation rare

Dilated superficial veins
Venous gangrene (v. rare)

12

Venous Thrombosis:Note

Size of clot doesn’t relate to symptoms

13

Venous Thrombosis: DVT diagnosis

Clinical history
Physical examination
Wells Score (screening) (2+) SEE MORE
D Dimer blood test (screening) – low good/
Confirmatory tests

14

Venous Thrombosis: Confirmatory tests

Doppler ultrasound
(Venography)
CTV/MRV for VTE at unusual

15

Venous Thrombosis: Doppler Ultrasound

Flow (red colour is not visible in the main vein (arrows), indicating lumen filled with thrombosis (SEE image)

16

Pulmonary Embolism: Symptoms


SOB
Cough
(Pleuritic) chest pain
Haemoptysis
Syncope
Palpitations
Sweating

17

Pulmonary Embolism: Diagnosis and results

Wells Score
ECG - Sinus tachycardia. R heart strain
ABG – Low O2/Co2
CXR – Usually normal potentially with wedge infarcts
V/Q scan – indeterminate in 50-70%
CT pulmonary angiogram - definitive

18

Pulmonary Embolism: DVT and PE sequelae

PE →
• Pulmonary Hypertension → Chronic PE
• Death
Deep Vein insufficiency
• Post-thrombotic syndrome
• Venous ulcers

19

Pulmonary Embolism: Management of VTE

Fast acting anticoagulation minimum 3 moths (LMW Heparin or rivaroxaban)
• PE with haemodynamic effect may need thrombolysis or thrombectomy
• DVT graded compression stocking for PTS (minimum 6 months post DVT)
Long term anticoagulation?
Depends on individualised risk vs. benefit

20

Pulmonary Embolism: Who is at risk of VTE

Genetic risk factors
Acquired risk factors:
• Immobility
• Trauma and surgery
• Pregnancy and peurperium (post natal care)
• Oestrogen therapy (e.g. COCP, HRT)
• Inflammatory disorder (e.g. IBD)
• Myeloproliferative disorders (e.g. Essential Thrombocythaemia)
• Malignancy (e.g. Adenocarcinoma)
• Antiphospholipid syndrome

21

Factor V Leiden: Definition

Sequence change in Factor V prevents inactivation by Protein C
5-10% Caucasians

22

Factor V Leiden: Epi

5-10% Caucasians
Found in 20% of individuals with unexplained thrombosis
FVL – increased risk of VTE x 4
COCP – increased risk of VTE x4
COCP + FVL – increased risk of VTE x16

23

Anti-phospholipid syndrome: Clinical features


CLOTs:
C: Arterial or Venous thrombosis
L: Livedo reticularis
O: Obstetric complications - Recurrent miscarriage
T: Thrombocytopaenia

24

Anti-phospholipid syndrome: Caused by

Antiphospholipid antibodies – bind to membrane phopspholipid glycoprotein complexes

25

Anti-phospholipid syndrome: Primary or Secondary to

Connective tissue disease (SLE)
Lympjoproliferative disorders (e.g. Lymphoma, CLL)
Infection
Drug induced

26

Anti-phospholipid syndrome: Antiphospholipid antibodies

Anti-cardiolipin antibodies
Anti-beta2 glycoprotein antibodies
Lupus anticoagulants
• Prolonged aPTT in the test tube
• Is definitely not physiological anticoagulant – lab artefact

27

Antithrombotic drugs

Anti-platelet agents
Anti-coagulants

28

Anti-platelet act to

Arterial: Inhibit arterial thrombosis (ACS, PVD, CVD)

29

Anti-coagulant drugs

Veins and low pressure vessels:
Inhibit coagulation pathway
Inhibit venous/low pressure thrombosis (DVT, PE, CVA in AF and mechanilca heart valves, CBP, dialysis)

30

UK Licensed anticoagulants

Inhibit production of thrombin:
• UF Heparin
• LMW heparin
• Warfarin
• Danaparoid
• Fondaparinux
• Bivalirudin
• Argatoban
• Apixiban
• Dabigatran
• Rivaroxaban

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