Bleeding Disorders Flashcards
What to remember when taking a bleeding Hx
- Has the patient really got a bleeding disorder?
- How severe is the disorder? (ie how appropriate is the bleeding)
- Pattern of bleeding
- Cognenital or acquired
- Mode of inheritance
Types of bleeding
- Bruising
- Epistaxis
- Post-surgical bleeding
- Menorrhagia
- Post-partum haemorrhage
- Post-trauma
How to categorise the pattern of bleeding
Platelet type of coagulation factor
If the platelets were at fault in a bleeding disorder what kind of bleeding would occur
- Mucosal
- Epistaxis
- Purpura
- Menorrhagia
- GI
If the coagulation factors were at fault in a bleeding disorder what kind of bleeding would occur
- Articular
- Muscle haematoma
- CNS
3 types of hereditary bleeding disorders
- Haemophilia A
- Hamophilia B
- Von Willebrand Disease
Difference between Haemophilia A + B
- Both X-linked
- With identical phenotype
- Just slightly different mutation
Haemophilia clinical features
- Haemarthrosis
- Muscle haematoma
- CNS bleeding
- Retroperitoneal bleeding
- Post surgical bleeding
Complications of haemophilia
- Synovitis
- Chronic haemophilic arthropathy
- Neurovascular compression ( compartment syndrome)
- Other bleeding issues like stroke
What investigations diagnose Haemophilia
- Prolonged APTT (activated partial thromboplastin time)
- Normal PT and BT
- Reduced factor VII or FIX
- Genetic analysis
Rx for bleeding diathesis in Haemophilia
- Coagulation factor replacement
- Tranexamic acid
- Desmopressin IV, PO or sublingual (DDAVP) [only in mild haemophilia A, no use if severe or haemophilia B]
How does desmopressin treat haemophilia
Stimulates the release of Von Willebrand factor + coagulant factor VIII
Management of haemophilia (no the bleeding diathesis)
- Splints
- Physiotherapy
- Analgesia
- Synovectomy
- Joint replacement
What is bleeding diathesis
Unusual succeptibility to bleed
What is bleeding diathesis
Unusual susceptibility to bleed
Complication of Desmopressin (DDAVP)
- MI
- Hyponatraemia in babies
What type of bleeding does Von Willebrand Brand disease cause
Platelet type (mucosal e.g. mouth, lips, tongue)
2 types of abnormalites of the VWF in von willebrand disease
- Quantitative
- Qualitative
3 types of Von Willebrand Disease
- Type 1 = Quantitative deficiency
- Type 2 = Qualitative deficiency
- Type 3 = severe (complete) deficiency
What investigations diagnose Von Willebrand disease
- Prolonged APTT (activated partial thromboplastin time)
- Increased BT
- Reduced factor VIII
- Reduced vWF Ag
What does vWF do
- Brings platelets into contact with exposed subendothelium
- Makes platelets bind to each other
- Binds platelets to factor VIII (protecting it from destruction in circulation)
Rx for Von Willebrand Disease
- vWF concentrate or Desmopressin (DDAVP)
- Tranexamic acid
5 types of acquired bleeding disorders
- Thrombocytopenia
- Liver failure
- Renal failure
- DIC
- Drugs (warfarin, heparin, aspirin, clopidogrel, rivaroxaban etc)
Causes of thrombocytopenia
- Decreased production (marrow failure)
- Increased consumption (DIC, hypersplenism, ITP)
How does ITP present and what cuases it
Immune Thrombocytopenia Purpura
- Usually in children
- Low platelet count, normal bone marrow, absence of other causes of thrombocytopenia
- Purpuric rash + increased tendency to bleed
- Caused by antiplatelet autoantibodies
4 types of bleeding seen in thrombocytopenia
- Petechia
- Ecchymosis (discoloration of skin due to bleeding underneath)
- Mucosal bleeding
- Rare CNS bleeding
What is Ecchymosis
Discoloration of skin due to bleeding underneath
What is Immune Thrombocytopenia Purpura (ITP) associated with
- Infection (esp. EBV, HIV)
- Collagenosis
- Lymphoma
- Drug induced
Who is mainly affected by chronic ITP and what are the symptoms
- Women
- Epistaxis + Menorrhagia
Rx of ITP
Only if symptomatic
- Prednisolone
- Thrombopoietin analohues (Eltrombopag)
- IV IgG
- Splenectomy (cures =80%)
- Immnosuppression if splenectomy fails (azathioprine)
Investigations to diagnose ITP
- Megakarocytes in marrow (?marrow biopsy?)
- Serum anitplatelet autoantibodies (?ELISA procedure?)
Changes related to bleeding in liver failure
- Reduced Factor I, II, V, VII, VIII, IX, X, XI
- Prolonged PT + APTT
- Reduced fibrinogen
Rx bledding disorders in liver failure
- Replacement FFP
- Vit K
Cause of haemorrhagic disease of the newborn (HDN)
Vitamin K deficient diet
Complication of HDN
Fatal or incapacitating haemorrhage
How to prevent HDN
Vitamin K IM/PO at birth
