12. Bleeding Disorders Flashcards
What forms the primary heamostasis
Platelet plug formation, platelets, vWF, wall
What forms the secondary heamostasic response
Fibrin
What is haemorrhaged diathesis?
Any quantitative or qualitative abnormality. Inhibition of function.
Can involve platelets, vWF, coagulation factors
What important points are important to investigate when taking a bleeding history
Has the patient actually got a bleeding disorder? How severe is the disorder Pattern of bleeding Congenital disorder? Acquired disorder? Mode of inheritance
How do you take a bleeding history?
Bruising Epistaxis Post surgical bleeding Mennorrhagia Post partum heamorrhage Post trauma
(Dental surgery, circumcision, tonsillectomy, appendectomy)
What classifies a severe bleeding disorder?
Spontaneous bleeding that occurs every 3/4 weeks. Can be into the body
What is platelet/mucosal bleeding
Caused by platelet factors or vFW deficiencies presents with
Epistaxis, purpurem mennorrhagia, GI
What is coagulation factor bleeding?
Bleeds into muscles, joints and articulate spaces
What are the features of mucosal bleeding?
Petichae, big bruises called e-something?
What is the classical presentation of people with coagulation disorders
Knee/ankle swelling. Hot, tender, swollen.
Coagulation factor 8 deficiency most common, called type 1 disorder.
How can you differentiate between congenital and acquires bleeding disorders?
Previous surgeries, did you bleed?
What is autosomal dominant inheritance?
Affected family members of each sex in each generation
What is hemophilia A?
Most common type of haemophilia, X linked, deficiency of coagulation factor 8. Severity of bleeding depends on the residual coagulation factor activity
What is heamophilia B
Christmas disease, arises from defect from coagulation factor 9. Again severity of bleeding depends on residual coagulation factor
What is the worst joint to be damaged by haemophilia?
Ankle
What are the clinical features of haemophilia?
Haemathrosis- blood in joint, tingles, throbs and becomes sore, unbearable pain Muscle heamatoma CNS bleeding Retroperitoneal bleeding Post surgical bleeding
What are the complications of haemophilia?
Synovitis
Chronic haemophillicnarthropathy
Neurovascular compression
Other sequelae of bleeding (stroke)
How do you diagnose haemophilia?
Clinical, prolonged APTT, normal PT reduced FVIII or FIX
Genetic analysis
How do you treat heamophilia?
Coagulation factor replacement
Entirely recombinant products
DDAVP- releases stored vFW and FVIII from endothelium
Tranexamic Acid- proheamostatic agent
Emphasis on prophalxysis
Gene therapy
Mezuzimab- monoclonal antibody- does the job of FVIII
What are the contraindication of DDAVP
Young children- hyponatraemia
People with cardiac complications
What is Von willebrands disease?
Autosomal dominant disease, typically mucosal bleeding.
How do you treat type I vWF
Tranexamic acid and DDAVP
vFW concentrate
Contraception- girls with menahhoregia
What is thrombocytopenia?
Decreased blood cells
This can be chased by marrow failure, aplasia and infiltration
Increased consumption causes immune ITP, non immune DIC and hypersplenism
What is the clinical pattern of thrombocytopenia
Petechia
EEhymosis
Mucosal bleeding
Rare CNS bleeding
Where’s the best place to look for petichae?
The ankles
What is ITP?
Seen in children post infection e.g. EBV
Can also been seen in lymphoma, drug induced or connective tissue disorders
How do you treat ITP?
Steroids, IV IgG, splenectomy
Thrombopoitein analogues
How does liver failure cause abnormalities in the blood?
Coagulation factors produced in liver as well as natural anticoagulants.
This leads to reduced fibrinogen and increase PT
Vit K dependant factors are also reduced
What is haemorrhaged disease of the newborn?
Every baby that is born has an immature coagulation system?
Vit K deficient diet causes heamorrhagic disease of the newborn
They get massive heamorrhags
