HAEM Flashcards
How is sickle cell disease inherited?
Autosomal recessive
If both parents had sickle cell trait, what is the odds of them having a child with sickle cell anaemia?
25%
If one parent had sickle cell anaemia and the other parent was not a carrier for sickle cell, what are the chances of them having a child with sickle cell anaemia AND sickle cell trait?
All of the children will have sickle cell trait
What does it mean to have sickle cell trait?
You carry the gene for sickle cell anaemia and could pass it on to your children, it very rarely causes any actual symptoms
What is the management for sickle cell crisis?
- analgesia (IV morphine)
- vigorous IV rehydration
- O2
- warming
- broad spectrum antibiotics
What is the management for chronic sickle cell disease? (4)
- Hydroxycarbamide
- penicillin prophylaxis
+ vaccine propylaxis due to hyposplenia
- 5 yearly pneumococcal vaccination
- single dose meningitis vaccination
How is thalassaemia inherited?
autosomal recessive
What is chronic lymphocytic leukaemia?
monoclonal proliferation of B cells
How does CLL present?
Often asymptomatic
- anorexia/ weight loss
- bleeding/ infections
- lymphadenopathy
What blood results would you see in FBC of someone with CLL? (3)
- lymphocytosis
- anaemia
- thrombocytopenia
What diagnosis would a finding of smear cells on blood film suggest?
CLL
What are 3 complications of CLL?
- hypogammaglobulinemia
- warm autoimmune haemolytic anaemia
- high grade lymphoma (Richters)
When does Chronic Myeloid Leukaemia typically present?
Age 60-70
Presence of the Philadelphia chromosome indicates which condition?
CML
What does the Philadelphia chromosome do?
Increases tyrosine kinase activity which increases cell proliferation