Bleeding Disorders Flashcards

1
Q

What is Haemophilia?

A

Bleeding Disorders resulting from inherited deficiency of a clotting factor.

A = Factor VIII

B = Factor IX

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2
Q

What is the inheritance pattern of Haemophilias?

A

X-Linked Recessive

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3
Q

How do Haemophilias present?

A

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

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4
Q

How do you diagnose a Haemophilia?

A

Prolonged APTT (Intrinsic Pathway)

Factor Assay shows low levels of factor (Diagnostic)

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5
Q

What is Von Willebrand Disease?

A

Reduced levels of effective vWF protein, due to

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

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6
Q

How does Von WIllebrand Disease present?

A

Superficial Bleeding

Bruising, Epistaxis, Menorrhagia

Gum Bleedin post-tooth extraction

Prolonged bleeding from minor wounds.

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7
Q

How would you investigate suspected Von Willebrand Disease`?

A

Increased APTT

Increased Bleeding Time

Decreased vWF levels

Normal PT

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8
Q

What is Disseminated Intravascular Coagulation?

A

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

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9
Q

How does DIC present?

A

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

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10
Q

How would you investigate a potential case of DIC?

A

FBC - thrombocytopaenia and Anaemia

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

Peripheral Blood Film shows Schistocytes

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