Anaemia Flashcards
Define haemolysis
Increased destruction of RBCs
What are haemolytic anaemias?
Group of anaemias in which red cell lifespan is reduced
How could a patient with haemolytic anaemia (low Hb) be treated?
Folic acid - increased requirement for erythropoiesis
Splenectomy (if severe) to increase red cell life span
What does an increase in unconjugated bilirubin indicate?
Problem with haemolysis of RBCs, a post-blood transfusion reaction, cirrhosis of the liver
What name is given to RBCs that are very round without the usual central pallor?
Spherocytyes
What is hereditary spherocytosis?
A disruption of vertical linkages in the red cell membrane causing red blood cells to become more spherical shaped
What do polychromatic macrocytes look like?
Larger and more blue than normal red cells
What are the four primary underlying mechanisms of anaemia?
reduced production of red cells/haemoglobin by the bone marrow
(b) reduced survival of red cells in the circulation (haemolysis).
(c) loss of blood from the body
(d) pooling of red cells in a very large spleen.
What are the symptoms associated with hereditary sopherocytosis?
Increased red cell osmotic fragility
Jaundice
Gall stones
What is seen on the blood film of someone with hereditary spherocytosis?
Spherocytes
Polychromatic macrocytes
High reticulocyte count
High unconjugated bilirubin
What test involving water can be done to test for hereditary spherocytosis?
Osmotic fragility test - reveals spherocytosis fragility in hypotonic solution
What does an increased unconjugated bilirubin suggest?
Pre-hepatic cause of jaundice
Caused by haemolysis
What does an increased conjugated bilirubin suggest?
Decreased elimination of bilirubin by hepatocytes
What are the common complications associated with haemolytic anaemia?
Gall stones
- increased breakdown of Hb to BR
- can lead to jaundice
How is hereditary spherocytosis treated?
Folic acid
Transfusion if severe
Splenectomy if severe but avoid if possible
What is the lab evidence for haemolysis/ haemolytic anaemia?
LDH inc
Unconjugated hyperbilirubinaemia
Reduced Haptoglobins
Increased MCV
Reticulocytosis
Acquired haemolytic anaemia is a result of what?
A problem in the RBC environment (plasma/ vasculature)
What environmental factors can damage RBCs?
•Environmental factors can damage RBCs
•Non-immune
> microangiopathic
> haemolytic uraemic syndrome
> malaria
> snake venom
> drugs
•Immune mediated
> auto immune
> allo immune (post blood transfusion)
Haemolytic anaemia can be categorised into having what 2 causes?
Acquired or inherited
Inherited haemolytic anaemia is the result of what?
A defect in RBC themselves
Wheat RBC defects can cause inherited haemolytic anaemia?
• abnormal cell membrane - eg. Hereditary spherocytosis
• abnormal Hb - eg. sickle cell
• defect in glycolytic pathway - eg. pyruvate kinase deficiency
• defect in enzymes of pentose shunt - eg. G6PD deficiency
How do we confirm wether the mechanism of haemolytic anaemia is immune or not ?
DAT: direct antiglobulin test
- autoantibodies to erythrocytes antigens can be detected
- a rabbit antibody to human immunoglobulin is added to the sample
- positive DAT confirms immune mechani
What is autoimmune haemolytic anaemia?
Patient develops auto-antibodies against their own RBCs via a Type II hypersensitivity reaction