Paeds Haematology Flashcards
Where are RBC’s broken down?
In the liver into bilirubin
What are the inherited causes of haemolytic anaemia?
- Hereditary spherocytosis: RBC’s are shaped like spheres
- Hereditary elliptocytosis: RBC’s are elliptical (oval) shape
- Sickle cell anaemia
- Thalassaemia: body doesn’t make enough Hb
- G6PD deficiency: causes haemolysis
- Pyruvate kinase deficiency: RBC’s break down faster than they should
What are some acquired causes of haemolytic anaemia?
- Autoimmune haemolytic anaemia
- Haemolytic transfusion reactions
- Paroxysmal nocturnal haemoglobinuria: autoimmune
- Infections: EBV, malaria
- Drugs
- Hypersplenism
What is the treatment for Hereditary spherocytosis?
Folic acid
Blood transfusions
Splenectomy (+cholecystectomy)
What is the management of thalassaemia?
- Blood transfusions
- Desferrioxamine: binding free iron or aluminium in the bloodstream and enhancing its elimination in the urine. By removing excess iron or aluminium, the agent reduces the damage done to various organs and tissues, such as the liver
- Bone marrow transplant
What contributes to G6PD deficiency?
- Fava bean: a variety of broad bean
- Dapsone: Used for leprosy and skin infections
- Nitrofurantoin
- Quinine: treats malaria
- Sulphonamides: antimicrobial
What is Marfan’s syndrome?
A connective tissue disorder
Features:
- Tall and slender build
- Disproportionately long arms, legs and finger
- Protruding breastbone
- A high, arched palate and crowded teeth
- Heart murmurs.
- Extreme near sightedness
- An abnormally curved spine
- Flat feet
What are the investigations for a child that bruises easily?
1st line
- FBC and blood film, PT time, APTT, fibrinogen, LFT
2nd line
- Von Willebrands factor activity & antigen, factor VIII, blood group
3rd line
- Full clotting factor assays, platelet function tests, bone marrow aspirate/trephine, refer to paediatric haematology unit
