Haematology 5 - Haemolytic anaemias Flashcards
Recall some causes of intravascular vs extravascular haemolytic anaemias
Intravascular: - Malaria (black water fever) - G6PDD - MAHA (due to HUS/TTP/PNH) Extravascular: - Autoimmune - hereditary spherocytosis
Which infection are patients with haemolytic anaemias more susceptibile to?
Parvovirus B19
What is the expected LDH level in haemolytic anaemia?
High
What is the inheritance pattern of hereditary spherocytosis?
Autosomal dominant
How can hereditary spherocytosis be diagnosed?
Examination of mean cell fluorescence
Osmotic fragility test
What is the difference in clinical features of hereditary elliptocytosis in a heterozygote vs a homozygote?
Heterozygote: no polychromasia
Homozygote: Severe haemolytic anaemia (pyropoikilocytosis)
In which parts of the world is G6PDD most common?
Where malaria is endemic
What is the inheritance pattern of G6PDD?
X linked recessive
What is the normal physiological role of G6PD?
NADPH generation
What is the main symptom of G6PDD in neonates?
Jaundice
Recall 3 triggers for a crisis in G6PDD
Moth balls
Fava beans
Anti-malarials
What is the typical appearance of erythrocytes in Pyruvate Kinase Deficiency?
Kinocytes (‘spiky’)
What is the first investigation to do in haemolytic anaemia?
DAT/ Coombs test
Need to exclude autoimmune haemolysis
What does a positive urinary haemosiderin test show?
Intravascular haemolysis
What is homozygous hereditary elliptocytosis also known as?
Pyropoikilocytosis