1B anaemia Flashcards
How do we interpret an increase in unconjugated bilirubin?
- Increased RBC breakdown
- Decreased liver function
- Increased Hb production
- Biliary obstruction
Describe any abnormalities in this blood film
Spherocytes: There are very round cells without the usual central pallor (which reflects the disc shape of a normal red cell).
What is hereditary spherocytosis?
Inherited defect in the RBC membrane.
There is disruption of vertical linkages in membrane, usually akyrin/spectrin.
What do we mean by the term ‘haemolysis’ and ‘haemolytic anaemia’?
- Haemolysis: increased destruction of RBCs (reduced RBC survival)
- Haemolytic anaemia: group of anaemias in which RBC lifespan is reduced (i.e. haemolysis that leads to a reduction in Hb)
How would a patient with haemolytic anaemia be treated?
- Folic acid (increased requirement for erythropoiesis)
- Splenectomy (if severe) to increase RBC life span
What can increased breakdown of haemoglobin to bilirubin cause?
- Gallstones
- Can obstruct CBD and cause obstructive jaundice
Compare conjugated and unconjugated bilirubin
How does the bone marrow respond to haemolytic anaemia?
- Increased EPO production
- Leads to reticulocytes
What is DAT (i)?
Direct antiglobulin test (i)
What is DAT (ii)?
Direct antiglobuin test (ii)
Extended or modified version of the DAT that includes additional testing parameters
What is a differential for DAT positive?
Auto-immune Haemolytic Anaemia (AIHA)
- Idiopathic
- Associated with disorders of immune system
- SLE
- Underlying lymphoid cancers (lymphoma)
What is laboratory evidence of haemolysis?
- LDH raised
- Unconjugated hyperbilirubinaemia
- Reduced haptoglobins
Acquired haemolytic anaemia is a result of RBC defect or RBC environment (plasma/vasculature) defect?
Environmental
Inherited is a result of RBC defect
What are environmental factors which can damage RBCs?
Non-immune
- Microangiopathic
- Haemolytic uraemic syndrome
- Malaria
- Snake venom
- Drugs
Immune mediated (DAT +ve)
- Auto-immune
- Allo-immune (post-blood transfusion)
What blood disorders are spherocytes seen in?
- Hereditary spherocytosis
- Acquired auto-immune haemolytic anaemia
What do the small arrows show?
Irregularly contracted cells
What do the larger arrows show?
There is a special test for Heinz bodies but in fact you can see them in a normal blood film
What do irregularly contracted cells, ghost cells and Heinz bodies indicate?
Oxidant damage to the red cell. Heinz bodies are precipitated oxidised haemoglobin.
Ghost cells show that there has been intravascular haemolysis
What should the patient be advised to do when confirmed with G6PD deficiency?
- Avoid oxidant drugs
- Don’t eat broad beans (fava beans)
- Avoid naphthalene
- Be aware that haemolysis can result from infection
What is G6PD?
An important enzyme in the hexose monophosphate (HMP) shunt, which is tightly coupled to the metabolism of glutathione, which protects red cells from oxidant damage.