B Lecture 10: Paediatric Haematology Flashcards
How should congenital leukaemia in Down’s syndrome be managed?
It will resolve spontaneously so it’s okay
Why may there be Howel-Jolly bodies on the blood film in sickle cell disease?
They are produced when there is splenic infarction
If not identified in a Guthrie spot, at what age does sickle cell disease tend to present?
6 months
In what age group might the hand-foot syndrome of sickle cell disease present?
<2 years
Why is there no risk of splenic sequestration in sickle cell disease once Howel-Jolly bodies have been identified on blood film?
Once there has been a splenic infarction (which will cause Howel Jolly bodies) you will get hyposplenism but there is no risk of sequestration
Recall 2 drugs that are required lifelong in all sickle cell disease patients?
Folic acid Penicillin (for protection against encapsulated bacteria because of hyposplenism)
In sickle cell disease, when is the highest risk of stroke?
In childhood (actually less common in adults with sickle cell)
What is the main risk of blood transfusions in treating thalassaemia?
Iron overload
Recall some inherited causes of haemolytic anaemia
SpherocytosisElliptocytosis PKU deficiencyG6PD deficiencySickle cell
What is the most common cause of acquired haemolytic anaemia in children?
E coli causing haemolytic uraemic syndrome
Which inherited defect of coagulation often presents with mucosal bleeding?
Von willebrand disease
How can you test for von willebrand disease?
Factor VIII assay
What is the treatment for von willebrand disease?
Low purity factor VIII
In which haemoglobinopathy is there benefit to carotid doppler monitoring?
Sickle cellDo doppler monitoring alongside exchange transfusion if there is turbulent carotid flow
