Haem: Paediatric haematology Pt.3

Question 1

How is AIHA diagnosed?

Answer 1

• Positive DAT
• Spherocytes on blood film

Question 2

What is the HUS triad?

Answer 2

• Acute renal failure
• Microangiopathic haemolytic anaemia
• Thrombocytopenia

Question 3

What is microangiopathic haemolytic anaemia?

Answer 3

Intravascular haemolysis of occuring due excessive shear forces in small vessels

Question 4

What can be seen on blood film that is pathognomic of MAHA?

Answer 4

Schisocytes

Question 5

What are the most common inherited defects of coagulation?

Answer 5

• Haemophilia A
• Haemophilia B
• Von Willebrand disease

Question 6

Describe the typical presentation of haemophilia A and B in an infant.

Answer 6

• Haemarthrosis when starting to walk
• Bruises
• Excessive post-traumatic or surgery bleeding

Question 7

List some differential diagnoses for haemophilia.

Answer 7

• Inherited thrombocytopaenia/platelet defect
• Acquired defects of clotting (e.g. ITP, acute leukaemia)
• Non-accidental injury
• Henoch-Schonlein purpura

Question 8

What are some key aspects of investigating a child with a suspected defect of coagulation?

Answer 8

• History and examination
• Family history
• Coagulation screen (APTT, PT, fibrinogen)
• Platelet count (rules out platelet causes)
• Assays for specific coagulation factors

Question 9

List some specific details of an infant’s early history that could be suggestive of a disorder of coagulation.

Answer 9

• Bleeding from the umbilical cord
• Bleeding after the Guthrie test
• Haematoma formation after vitamin K injection/vaccines
• Bleeding after circumcision

Question 10

What are the principles of treatment of inherited disorders of coagulation?

Answer 10

• Counselling the family
• Treatment of bleeding episodes
• Use of prophylactic coagulation factors

Question 11

Describe the typical presentation of von Willebrand disease.

Answer 11

• Mucosal bleeding
• Bruises
• Post-traumatic bleeding

Question 12

Why do von Willebrand disease and haemophilia A present similarly?

Answer 12

They are both characterised by low level of factor 8

Question 13

How is von Willebrand disease diagnosed?

Answer 13

• Family history (mainly autosomal dominant)
• Coagulation screen
• vWF antigen assay
• Factor 8 assay
• (Bleeding time)
• Platelet aggregation studies

Question 14

How is von Willebrand disease treated?

Answer 14

• Desmopressin (stimulates vWF release from endothelium)
• Factor VIII or vWF replacement

Question 15

Describe the relative prevalence of haemophilia A and B.

Answer 15

Haemophilia A is 4x more common than haemophilia B

Question 16

Describe the typical presentation of ITP.

Answer 16

• Petechiae
• Bruises
• Bleeding from mucous membranes

Typically post-infection

Question 17

List some differential diagnoses for ITP.

Answer 17

• Henoch-Scholein Purpura
• Non-accidental injury
• Coagulation factor defect
• Inherited thrombocytopaenia
• Acute leukaemia

Question 18

How is ITP diagnosed?

Answer 18

• History
• FBC and blood film (low platelets with normal to large platelets on film)
• Coagulation screen (normal)
• Bone marrow biopsy (rarely)

Question 19

List some treatment approaches for ITP.

Answer 19

• Observation (most common)
• Corticosteroids
• High dose IVIG
• IV anti-RhD (if RhD positive)

Question 20

Which type of leukaemia is most common in children?

Answer 20

ALL

NOTE: < 1 years old AML is more common than ALL