Bleeding Disorders Flashcards
Normal haemostastic response
Primary- Platelet Plug formation, platelets, vWF, endothelial wall
Secondary- Fibrin plug formation
What is haemorrhagic diathesis?
Unusual susceptibility to bleed
- Any quantitative or qualitative abnormality inhibition of function
- Due to platelets, vWF, coagulation factors
Points to ask in bleeding disorders bleeding history?
Has patient got a bleeding disorder, How severe is it?, pattern of bleeding, Congenital or acquired, Mode of inheritance
Key points to ask about with suspected bleeding disorder to ascertain how severe?
How appropriate? Bruising, Epistaxis, Post surgical bleeding (how long? Outcome? Dental surgery, tonsillectomy?) Menorrhagia, Post-partum haemorrhage, Post-trauma, Spontaneously?
If due to platelets what types of bleeds are you likely to see?
Mucosal Epistaxis Purpura Menorrhagia GI
If due to coagulation factors what types of bleeds are you likely to see?
Articular
Muscle haematoma
CNS (intracranial bleed)
Questions to ask to assess if bleeding disorder is congenital or acquired
Previous episodes ?
Age at first visit for bleed ?
Previous surgical challenges ?
Associated history ?
Types of inheritance of haemophilia
X-linked
What is haemophilia?
Genetic disorder impairing body’s function to make clots
Severity of bleeding depends on the residual coagulation factors activity:
<1% severe
1-5% moderate
5-30% mild
Presentation of haemophilia
Haemoarthrosis: Bleeding into joint
Muscle Haematoma: Bleeding into muscle
CNS bleeding: Less commonly seen
Retroperitoneal bleeding
Post-surgical bleeding
Difference between haemophilia A and B
A=deficiency in factor 8
B=deficiency in factor 9
Haemophilia clinical complications
Synovitis,
Chronic haemophilic arthropathy,
Neurovascular compression (eg compartment syndromes),
Other sequelae of bleeding (eg stroke)
Haemophilia diagnosis
Clinical, Prolonged APTT, Normal PT, Reduced factor 8 or 9, Genetic analysis
Haemophilia treatment of bleeding diathesis
Coagulation factor replacement, DDAVP (causes release of vWF) Tranexamic acid, Prophylaxis, Gene therapy
Treatment of haemophilia to prevent bleeds
Splints, Physiotherapy, Analgesia, Synovectomy, Joint replacement
Haemophilia treatment complications
Viral infection- HIV, HBV, HCV
Inhibitors- anti FVIII antibodies
DDAVP- MI, hyponatraemia
Typical clinical picture of haemophilia
Male
6mnts-1yr
Bleeding into joint
What is the inheritance type of Von Willebrand Disease
Autosomal dominant
Presentation of Von Willebrand Disease
Mucosal pattern of bleeding: Easily bruising, Large bruises, Frequent/prolonged epistaxis, Bleeding gums, Heavy/prolonged bleeding from cuts, Menorrhagia, Post partum haemorrhage
Von Willebrand Disease types
Type I:
Mildest and most common form
Quantitative defect
Don’t make enough
Type II (A,B,M,N):
Qualitative deficiency
Quality of the protein is not good enough
Doesn’t work properly
Type III:
Severe
Complete deficiency
Treatment of Von Willebrand Disease?
vWF concentrate or DDAVP
Tranexamic acid
Topical applications
OCP
Causes of acquired bleeding disorders
Thrombocytopenia, Liver failure, Renal failure, DIC, Drugs- warfarin, heparin, aspirin, clopidogrel, rivaroxaban, apixaban, dabigatran, bivalirudin
Causes of thrombocytopenia
Decreased production of platelets:
- marrow failure
- aplasia
- infiltration
Increased consumption of platelets:
- immune ITP
- non immune DIC
- hypersplenism
Thrombocytopenia clinical presentation
Petechia,
Ecchymosis,
mucosal bleeding,
Rare CNS bleeding
What does ITP stand for
Immune Thrombocytopenic purpura
What is Immune thrombocytopenia purpura?
Defined as low platelet count with normal bone marrow
And absence of other causes of thrombocytopenia
2 distinct patterns: Acute and Chronic
Describe immune ITP
Caused by antiplatelet antibodies
Acute:
Children
Following acute infection
Self-limiting purpura
Chronic:
Adults
Mainly women
Fluctuating course
What is associated with ITP
Infection esp. EBV, HIV
Collagenosis
Lymphoma
Drug induced esp quinine
Treatement for ITP
Steroids,
IV IgG,
Splenectomy,
Thrombopoietin analogues (Eltrombopag, romiplostim)
What coagulation factors are associated with liver
And diagnostic findings of liver failure with regards to bleeding?
Factor I, II, VII, IX, X, XI
Prolonged PT and APTT
Reduced fibrinogen
Treatment of bleeding in liver failure
FFP,
Vitamin K
Treatment of haemorrhagic disease of newborn
Vitamin K !!
