SYMPOSIUM: Sickle cell anaemia

Question 1

What is dactylitis?

Answer 1

Severe inflammation of fingers and joints causing rigidness

Question 2

What are the clinical features of chest syndrome of sickle cell anaemia?

Answer 2

SOB, pleuritic chest pain, patchy shadowing on a CXR, progressive hypoxia and fever

Question 3

What are the clinical features of brain syndrome of sickle cell anaemia?

Answer 3

Presents as a stroke

Question 4

What is an aplastic crisis?

Answer 4

When the body doesn’t make enough RBC’s. It’s associated with parvovirus infection

Question 5

What is hydroxyurea/hydroxycarbamide?

Answer 5

A medication used to reactivate gamma globin locus in patients that leads to an increase in production of foetal Hb. This results in reduced sickling of cells and reduces crisis

Question 6

What is HPFH (Hereditary persistence of foetal Hb)?

Answer 6

People who have Foetal Hb levels over 20%. These people have less crisis with sickle cell anaemia

Question 7

What is allogeneic SCT?

Answer 7

A procedure in which a patient receives healthy blood-forming stem cells