3: Haemolytic Anaemia Flashcards
What is haemolytic anaemia
pre-mature destruction of RBC
What is the lifespan of a RBC
120-days
What are the two types of haemolytic anaemia
Intravascular haemolysis
Extravascular haemolysis
Where does intravascular haemolysis occur
Inside blood-vessels
Where does extravascular haemolysis occur
Outside blood-vessels
Aside from intravascular-extravascular how can anaemia be divided
Hereditary
Acquired
What are the 2 hereditary haemolytic anaemia due to enzyme defects
- G6PD Deficiency
- Pyruvate Kinase Deficiency
What are the 2 hereditary haemolytic anaemia due to membrane defects
- Hereditary spherocytosis
- Hereditary elliptocytosis
What are the 2 haemolytic anaemia due to haemaglobinopathies
- Thalasemia
- Sickle cell
How can acquired haemolytic anaemias be divided
Immune mediated and non-immune mediated
If an immune mediated haemolytic anaemia, what will be positive
Coombs test (Direct antibody test)
what is a group of Coombs negative acquired haemolytic anaemia
Microangiopathic haemolytic anaemia
What is the inheritance pattern of G6PD deficiency
X-Linked recessive
How does G6PD present normally
Asymptomatic
What can precipitate a crisis in G6PD deficiency
- anti-malarial
- sulphur containing drugs: sulphonylurea, sulphasalazine,
- ciprofloxacin
- fava beans
How will G6PD crisis present
Jaundice
Gall stones
Splenomegaly
How may G6PD manifest in early days of life
Neonatal Jaundice
what is the most common RBC enzyme defect
G6PD Deficiency
which gender is G6PD more common in and why
Males - due to x-linked recessive
explain pathophysiology of G6PD
G6PD reduces glutathione. Deficiency therefore increases glutathione and oxidative stress leading to RBC breakdown and intravascular haemolysis