Haemoglobinopathies Flashcards

1
Q

Adult Hb consists of what?

A

2 alpha

2 beta chains

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Foetal Hb consists of what?

A

2 alpha

2 gamma chains

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Which ethnicity is sickle cell disease common in?

A

Afro-Caribbeans

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is sickle cell disease?

A

Mutation of the beta globin gene resulting in a HbS variant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

How is sickle cell disease inherited?

A

Autosomal recessive disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

How would a sickle cell patient’s RBCs appear on a blood film?

A

Sickle shaped

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

How would you manage sickle cell crisis? (5)

A
Analgesia
Oxygen to achieve target stats
IV fluids
Antibiotics
Blood transfusion
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are the acute complications of sickle cell anaemia? (4)

A

Vaso-occlusive crisis
Stroke/ TIA
Infection
Acute chest syndrome (chest pain, cough, lung infiltrates)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are the chronic complications of sickle cell anaemia? (3)

A

Pulmonary hypertension
Joint damage
Renal disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are the disease-modifying treatments for sickle cell anaemia? (3)

A

Hydroxycarbamide (increases HbF conc)
Bone marrow transfusion (risk of iron overload)
Stem cell transplant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is thalassaemia?

A

Hereditary diseases causing faulty production & premature destruction of RBCs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Thalassaemia is common in which continents? (3)

A

Mediterranean
Middle East
Asia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly