Bleeding Disorders Flashcards
What is Haemophilia?
Bleeding Disorders resulting from inherited deficiency of a clotting factor.
A = Factor VIII
B = Factor IX
What is the inheritance pattern of Haemophilias?
X-Linked Recessive
How do Haemophilias present?
Early in life or after Surgery/trauma
Haemarthrosis after minimal truama - Painful Joints
Haematomas - Painful bleeding into muscles
Excess bleeding and bruising
Haematuria
OE - Bruises, joint deformities, IDA
How do you diagnose a Haemophilia?
Prolonged APTT (Intrinsic Pathway)
Factor Assay shows low levels of factor (Diagnostic)
What is Von Willebrand Disease?
Reduced levels of effective vWF protein, due to
1) Reduced levels
2) Defective protein
3) Complete lack of vWF and reduced Factor VIII
How does Von WIllebrand Disease present?
Superficial Bleeding
Bruising, Epistaxis, Menorrhagia
Gum Bleedin post-tooth extraction
Prolonged bleeding from minor wounds.
How would you investigate suspected Von Willebrand Disease`?
Increased APTT
Increased Bleeding Time
Decreased vWF levels
Normal PT
What is Disseminated Intravascular Coagulation?
Emergency condition characterised by the formation of Fibrin clots in the body’s microcirculation, leading to red cell fragmentation (MAHA).
How does DIC present?
Severely unwell patient with a history of underlying disease and evidence of bleeding
Fever, Shock, Oliguria
Sepsis
Acute: Petechiae, Purpura, Ecchymoses, Epistaxis, Mucosal Bleeding, Respiratory Distress
How would you investigate a potential case of DIC?
FBC - thrombocytopaenia and Anaemia
Clotting Profile - Decreased Fibrinogen, Increased PT/APTT, Increased Fibrin Degradation Products
Peripheral Blood Film shows Schistocytes