Bleeding Disorders

Question 1

What is Haemophilia?

Answer 1

Bleeding Disorders resulting from inherited deficiency of a clotting factor.

A = Factor VIII

B = Factor IX

Question 2

What is the inheritance pattern of Haemophilias?

Answer 2

X-Linked Recessive

Question 3

How do Haemophilias present?

Answer 3

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

Question 4

How do you diagnose a Haemophilia?

Answer 4

Prolonged APTT (Intrinsic Pathway)

Factor Assay shows low levels of factor (Diagnostic)

Question 5

What is Von Willebrand Disease?

Answer 5

Reduced levels of effective vWF protein, due to

1) Reduced levels
2) Defective protein
3) Complete lack of vWF and reduced Factor VIII

Question 6

How does Von WIllebrand Disease present?

Answer 6

Superficial Bleeding

Bruising, Epistaxis, Menorrhagia

Gum Bleedin post-tooth extraction

Prolonged bleeding from minor wounds.

Question 7

How would you investigate suspected Von Willebrand Disease`?

Answer 7

Increased APTT

Increased Bleeding Time

Decreased vWF levels

Normal PT

Question 8

What is Disseminated Intravascular Coagulation?

Answer 8

Emergency condition characterised by the formation of Fibrin clots in the body’s microcirculation, leading to red cell fragmentation (MAHA).

Question 9

How does DIC present?

Answer 9

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

Question 10

How would you investigate a potential case of DIC?

Answer 10

FBC - thrombocytopaenia and Anaemia

Clotting Profile - Decreased Fibrinogen, Increased PT/APTT, Increased Fibrin Degradation Products

Peripheral Blood Film shows Schistocytes