Bleeding disorders

Question 1

Important history questions when confronted with a potential bleeding disorder

Answer 1

• is the child a bleeder?
• what type of bleeding is it?
• how severe is the disorder?
• is it inherited?

Question 2

How do you know if the child is a bleeder?

Answer 2

• bruises at abnormal sites
• any spontaneous bleeding
• bleeding disproportionate to the degree of trauma
• prolonged bleeding from cuts
• haemorrhage following dental extraction/surgery

Question 3

Factors that promote normal haemostasis

Answer 3

• vascular contraction
• platelet aggregation
• coagulation of blood

Question 4

When does foetal blood start to clot?

Answer 4

11 weeks

Question 5

How does a thrombocytopenia present:?

Answer 5

• epistaxis
• mucus membranes
• petechiae
• small bruises
• GIT
• menorrhagia

Question 6

How do coagulation defects present?

Answer 6

• deep seated haematomas
• haemarthrosis
• renal

Question 7

Definition of purpura

Answer 7

• bleeding into skin or mucous membranes

Question 8

Types of purpura

Answer 8

• thrombocytopaenic purpura

- vascular purpura (non-thrombocytopaenic)

Question 9

Examples of X-linked bleeding disorders

Answer 9

• haemophilia A and B

- Wiskott Aldrich syndrome

Question 10

Examples of autosomal dominant bleeding disorders

Answer 10

• von Willebrand disease

- Factor XI deficiency

Question 11

Examples of autosomal recessive bleeding disorders

Answer 11

• other factos deficiencies

- platelet function disorders

Question 12

Screening tests for bleeding disorders

Answer 12

• platelet count
• INR
• PTT
• bleeding time

Question 13

Causes of thrombocytopaenia

Answer 13

• diminished production
• peripheral consumption
• platelet sequestration
• ineffective thrombopoeisis

Question 14

Congenital disoders of platelet function

Answer 14

• thrombasthaenia
• storage pool disease
• abnormal release mechanism
• Bernard-Soulier syndrome

Question 15

Acquired disorders causing defective platelet funciton

Answer 15

• scurvy
• uraemia
• drugs
• collagen disorders
• MDS and leukaemia
• hypothyroidism
• viral infections

Question 16

Pattern of von Willbrand disease bleeding

Answer 16

• mucocutaneous

- post-traumatic and post-surgical

Question 17

Lab features of von Willebrand disease

Answer 17

• prolonged BT
• prolonged PTT
• decreased vWF
• decreased VIII

Question 18

Differentials for bleeding in the newborn

Answer 18

Coagulation defects

• haemorrhagic disease of the newborn
• DIC
• haemophilia

Platelet deficiencies

• thrombocytopaenia
• function defects