Haematology Coagulation 2 Flashcards
(76 cards)
Haemophilia: Description
Defect in Factors that affect clotting factors downstream
Haemophilia A
Defect in F8 gene causing reduced FVIII
Haemophilia B
Defect in F9 causing reduced FIX
Haemophilia: Inheritance
Sex Linked – FIND OUT MORE
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
Haemophilia: Treatment
Recombinant factor concentrate – personalised prophylaxis regimes to ensure factor levels never drop to ‘severe’ levels
→ Implant venous access device for easier infusions
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
Venous Thrombosis: Thrombophilia
Increased propensity to VTE
Acquired + genetic risk factors
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
Venous Thrombosis:DVT presentation common
Unilateral pain
Swelling
Tenderness
Discolouration
Venous Thrombosis: DVT presentation rare
Dilated superficial veins Venous gangrene (v. rare)
Venous Thrombosis:Note
Size of clot doesn’t relate to symptoms
Venous Thrombosis: DVT diagnosis
Clinical history Physical examination Wells Score (screening) (2+) SEE MORE D Dimer blood test (screening) – low good/ Confirmatory tests
Venous Thrombosis: Confirmatory tests
Doppler ultrasound
(Venography)
CTV/MRV for VTE at unusual
Venous Thrombosis: Doppler Ultrasound
Flow (red colour is not visible in the main vein (arrows), indicating lumen filled with thrombosis (SEE image)
Pulmonary Embolism: Symptoms
SOB Cough (Pleuritic) chest pain Haemoptysis Syncope Palpitations Sweating
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
Pulmonary Embolism: DVT and PE sequelae
PE → • Pulmonary Hypertension → Chronic PE • Death Deep Vein insufficiency • Post-thrombotic syndrome • Venous ulcers
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
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
Factor V Leiden: Definition
Sequence change in Factor V prevents inactivation by Protein C
5-10% Caucasians
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
Anti-phospholipid syndrome: Clinical features
CLOTs: C: Arterial or Venous thrombosis L: Livedo reticularis O: Obstetric complications - Recurrent miscarriage T: Thrombocytopaenia
Anti-phospholipid syndrome: Caused by
Antiphospholipid antibodies – bind to membrane phopspholipid glycoprotein complexes
