Case 6 - Clotting Disorders Flashcards
What is the difference between PT and PTT?
PT - measures the extrinsic coagulation pathway
PTT - measures the intrinsic coagulation pathway and common coagulation pathway
What are the normal values for PT and PTT?
PT: 10-12 seconds
PTT: 20-30 seconds
Which clotting factors are involved the extrinsic and intrinsic pathways?
Extrinsic - V, VII, X
Intrinsic - VIII, IX, X, XI, XIII (Also Von willebrand factor)
What do APTT mixing studies show?
If APTT is raised then mix patient plasma with normal plasma (50:50 ratio)
If APTT time fails to correct - suggests coagulation factor inhibitor present (e.g, acquired antibody)
If APTT time corrects itself - suggests coagulation factor deficiency
What are the two types of haemophilia and which clotting factors are they deficient in?
Haemophilia A - factor VIII deficiency
Haemophilia B - factor IX deficiency
What is Von Willebrand disease and when would you suspect it?
Clotting disorder characterised by platelet deficiency and factor VIII deficiny
Suspect when history of bleeding but both PT and pTT are normal
What are the 3 types of Von willebrand disease?
1) mild, quantitative deficiency, partial reduction in vWF (80% of patients)
2) mild-moderate, qualitative deficiency, abnormal form of vWF
3) severe, quantitative deficiency, total lack of vWF (autosomal recessive)
What is the diagnostic triad for bleeding disorders?
1) personal history - bruising, bleeding, nosebleeds
2) family history - any family history of clotting disorders
3) diagnostic tests - PT, PTT, FBC, factor assays, LFTs
What is disseminated intravascular coagulation?
A condition in which there is an over activity of clotting factors causing small blood clots to develop throughout the bloodstream
What is virchows triad?
The 3 primary abnormalities that lead to thrombus formation
Abnormalities of the vessel wall - endothelial injury
Abnormalities of blood flow - statis or turbulent blood flow
Abnormalities of the blood’s constituents - hypercoagulability of the blood
What is iodiopathic thrombocytopenia (ITP)?
Excessive bleeding disorder due to low levels of platelets
When would you initiate platelet transfusion in a bleeding patient?
Platelet count <30x10^9 for significant bleeding or haematemesis, melena, prolonged epistaxis
Platelet count <100x10^9 for patients with severe bleeding or bleeding at critical sites such as the CNS
What is factor V Leiden
Clotting disorder - where the body clots too much
Due to mutation in factor V making it less likely to be cleaved by protein C
What score on the two level DVT Wells score?
Active cancer
Paralysis or recent plaster immobilisation of lower extremities
Recently bedridden for >3days, or major surgery within 12 weeks requiring general or regional anaesthesia
Localised tenderness along distribution of deep venous system
Entire leg swollen
Calf swelling on symptomatic side
Pitting oedema on symptomatic side
Collateral superficial veins (non varicose)
Previous DVT
How would you respond to the scoring of the wells score?
> 2 points (DVT likely) - proximal leg ultrasound within 4 hours
< 2 points (DVT unlikely) - D dimer test, if D dimer test positive then arrange proximal leg ultrasound within 4 hours
If proximal leg ultrasound cannot be carried out within 4 hours then low molecular weight heparin should be administered whilst waiting (it then should be carried out within 24 hours)
How is DVT or PE treated?
LMWH or fondaparinux given immediately and continued for at least 5 days or until INR is 2.0 or above for at least 24 hours
Vitamin K antagonist (Warfarin) - given within 24 hours and continued for at least 3 months
What is the mechanism of action of dabigatran?
Direct thrombin inhibitor