Case 6 - Clotting Disorders Flashcards
What are the disorders of the primary haemostasis?
- Abnormal platelet count
- Abnormal platelet time
What are the disorders of the secondary haemostasis?
- Intrinsic pathway: haemophilia A or haemophilia B
- Extrinsic pathway: factor VII deficiency
- Both pathways (Deficiency or inhibition of vitamin K-dependent coagulation factors II, VII, IX, and X AND Vitamin K antagonist therapy)
- What is the prothrommbin time?
- Which pathway is it used in?
- What can prolong it?
- Time it takes for the extrinsic system to produce fibrin polymers
- Extrinsic + common
- Warfarin, Factor II, V, VII and X deficiency (due to vit K def)
- What is INR?
- Which pathway is it used in?
- What is its clinical relevance?
- Comparing the lab specific prothrombin time to a standardized prothrombin time
- Normal: 1 .0
- Extrinsic + common
- Vitamin K deficiency or warfarin therapy monitoring
- What is the activated partial thromboplastin time (aPTT)?
- Which pathway is it used in?
- What can prolong it?
- Time it takes for the intrinsic system to produce fibrin polymers
- Intrinsic + common
- Heparin, Factor VIII, IX, XI, XII (but no bleeding diathesis) and
Von Willebrand’s Disease
- What is the thrombin time (TT)?
- Which pathway is it used in?
- What is its clinical relevance?
- Time it takes for fibrin polymers to form after adding thrombin
- Common
- Detect fibrinogen deficiency
What can be the reasons why PT and APTT are prolonged?
- Vitamin K deficiency: Liver disease, malabsorption
- DIC
- Rarely: Factor V or Factor X deficiency
What is disseminated intravascular coagulation?
Condition where procoagulant pathways are abnormally active resulting in formation of blood clots in small blood vessels throughout the body. Often associated with trauma, shock, and sepsis.
What are vitamin K dependant factors?
- Factor II
- Factor VII
- Factor IX
- Factor X
- Protein C
- Protein S
- What is 50:50 mixture test?
- When it is done?
- What do the results mean?
- When patient has abnormal PT or APTT, can test with mixed 50:50 patient’s blood and normal plasma
- If corrected, there is a deficiency of a Factor
- If uncorrected, there is an inhibitor of the coagulation factor.
What is VWD?
- Due to either a quantitative or qualitative abnormality of von Willebrand factor (VWF).
- It provides the critical link between platelets and exposed vascular sub endothelium
What inheritance pattern does VDW carry?
Autosomal dominant
What is type 1 VWD?
- Quantitative deficiency of VWF
- Mild disease, with some bruising/mucosal bleeding, menorrhagia and caution during dental extractions/surgery
What is type 2 VWD?
- Qualitative deficiency: usually underactive apart from rare type 2b in which it is overactive.
- Similar to Type 1 clinically.
- Avoid desmopressin because it will release VWF from stores, use VWF concentrate instead.
What is type 3 VWD?
- Little or no VWF
- Autosomal recessive trait
- Severe illness, with severe mucosal and joint bleeding.
- Extreme caution in surgery
What are the symptoms of VWD?
- Bleeding from minor wounds
- Post-operative bleeding
- Easy and excessive bruising (ecchymosis) – large bruises or bruising on the trunk or other areas
- Menorrhagia
- Epistaxis, GI bleeding
- Bleeding gums with brushing
- Petechiae: result of bleeding under the skin (small non raised patches)
What are the investigations for VWD?
- Normal PT
- APTT – Prolonged only if factor VIII is decreased sufficiently (less than 35% of normal). Often normal in T1 + 2.
- Decreased factor VIII levels: binds to vWF in blood to
- Von Willebrand factor antigen
How can Von Willebrand factor antigen be used to differentiate between type 1 and 2?
- If vWF activity: vWF antigen >0.6, then it is Type 1
- If vWF activity: vWF antigen <0.6, then it is Type 2
What is the treatment for VWD?
- Desmopressin can be used to stimulates the release of VWF (not used in type 3)
- VWF infusion
- Factor VIII infusion
- Antifibrinolytic drugs (aminocaproic acid and tranexamic acid): slows down break down of clots
What can be given to women with menorrhagia in VWD?
- OCP
- Antifibrinolytic drugs (aminocaproic acid and tranexamic acid): slows down break down of clots
What is haemophilia?
Bleeding disorder which results from the deficiency of a coagulation factor.
What is haemophilia A?
Deficiency of clotting factor VIII (80% of cases)
What is haemophilia B?
Deficiency of clotting factor IX (20% of cases)
What is the inheritance pattern for haemophilia?
- X-linked recessive
- Only men affected, females carriers
What are the symptoms of haemophilia?
- Bleed excessively in response to minor trauma
- Risk of spontaneous haemorrhage without any trauma
- Excessive bleeding after surgery, dental procedure
- Mucocutaneous bleeding: Easy bruising, GI bleeding, haematuria, epistaxis
- Musculoskeletal bleeding: pain and swelling (commonly extremities)
- Hemarthrosis: swelling of the joints (commonly knees, elbow and ankle) with pain