Sickle cell anaemia Flashcards
What kind of anaemia is sickle cell anaemia?
Haemolytic anaemia
Briefly describe the pathophysiology of sickle cell anaemia
Red blood cells are sickle (crescent) shaped, so are fragile and more easily broken down
The genetic mutation causing sickle cell anaemia is inherited in a (…?) pattern
Autosomal recessive
Having one copy of the sickle cell gene mutation is known as…
Sickle cell trait
Sickle cell trait reduces the severity of malaria infection… True or false?
True
When is sickle cell anaemia tested for?
- Pregnancy (in women at risk of being carriers)
- Newborn heel prick test
What are the 4 sickle cell crises?
- Vaso-occlusive crisis (AKA painful crisis)
- Splenic sequestration crisis
- Aplastic crisis
- Acute chest syndrome
What can trigger a sickle cell crisis?
- Cold
- Dehydration
- Infection
Briefly describe the pathophysiology of vaso-occlusive crisis
Sickle-shaped red blood cells block capillaries causing distal ischaemia (painful)
Briefly describe the pathophysiology of splenic sequestration crisis
Sickle-shaped red blood cells block blood flow within the spleen, causing an acutely enlarged an painful spleen
Briefly describe the pathophysiology of aplastic crisis
What is the most common trigger?
Temporary loss of creation of new blood cells, leading to significant anaemia
Most common trigger is parvovirus B19
A diagnosis of acute chest syndrome requires which two criteria?
1) Fever or respiratory symptoms
2) New infiltrates seen on CXR
Describe the general management of sickle cell anaemia
Generally supportive:
- Ensure vaccinations up to date
- Antibiotic prophylaxis
- Blood transfusions to treat anaemia
Which intervention can be curative in sickle cell anaemia?
Bone marrow transplant
What is the diagnostic investigation for sickle cell anaemia?
Hb electrophoresis
