Haematology 2 - Paediatric haematology Flashcards

1
Q

How should congenital leukaemia in Down’s syndrome be managed?

A

It will resolve spontaneously so it’s okay

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2
Q

Why may there be Howel-Jolly bodies on the blood film in sickle cell disease?

A

They are produced when there is splenic infarction

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3
Q

If not identified in a Guthrie spot, at what age does sickle cell disease tend to present?

A

6 months

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4
Q

In what age group might the hand-foot syndrome of sickle cell disease present?

A

<2 years

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5
Q

Why is there no risk of splenic sequestration in sickle cell disease once Howel-Jolly bodies have been identified on blood film?

A

Once there has been a splenic infarction (which will cause Howel Jolly bodies) you will get hyposplenism but there is no risk of sequestration

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6
Q

Recall 2 drugs that are required lifelong in all sickle cell disease patients?

A

Folic acid

Penicillin (for protection against encapsulated bacteria because of hyposplenism)

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7
Q

In sickle cell disease, when is the highest risk of stroke?

A

In childhood (actually less common in adults with sickle cell)

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8
Q

What is the main risk of blood transfusions in treating thalassaemia?

A

Iron overload

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9
Q

Recall some inherited causes of haemolytic anaemia

A
Spherocytosis
Elliptocytosis 
PKU deficiency
G6PD deficiency
Sickle cell
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10
Q

What is the most common cause of acquired haemolytic anaemia in children?

A

E coli causing haemolytic uraemic syndrome

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11
Q

Which inherited defect of coagulation often presents with mucosal bleeding?

A

Von willebrand disease

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12
Q

How can you test for von willebrand disease?

A

Factor VIII assay

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13
Q

What is the treatment for von willebrand disease?

A

Low purity factor VIII

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14
Q

In which haemoglobinopathy is there benefit to carotid doppler monitoring?

A

Sickle cell

Do doppler monitoring alongside exchange transfusion if there is turbulent carotid flow

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