Haemolytic anaemia

Question 1

What is haemolytic anaemia?

Answer 1

Destruction of RBC

Question 2

What are the causes?

Answer 2

Acquired

• autoimmune haemolytic anaemia
• alloimmune haemolytic anaemia (transfusion reaction and haemolytic disease of newborn)
• paroxysmal nocturnal haemoglobinuria (haematuria)
• prosthetic valve induced haemolysis

Congenital

• thalassaemia
• sickle cell
• G6PD deficiency
• hereditary spherocytosis

Question 3

What are the symptoms?

Answer 3

Anaemia
Jaundice
Splenomegaly

Can get gallstones

Question 4

What investigations are needed?

Answer 4

FBC- anaemia
Blood film
-spherocytes- HS
-ellipses- ellipsocytosis 
-sickle cells
-heinz bodies- G6PD deficiency

Coombs test- positive which shows antibodies against RBC

Question 5

What is hereditary spherocytosis?

Answer 5

Autosomal dominant condition
Production of sphere RBC which are fragile and rapidly broken down by spleen

Causes jaundice, splenomegaly, anaemia and gallstones
Can get aplastic crises with parvovirus infection
Raised reticulocytes

Treat with folate and splenectomy

Ellipsocytosis is the same but RBC in ellipse shape

Question 6

What is G6PD deficiency?

Answer 6

Defect in G6PD

Due to

• fava beans/broad beans
• anti malarials
• sulphonylureas
• sulfalazine

X linked recessive

Treat with precipitant avoidance and transfusion is severe

Question 7

What is autoimmune haemolytic anaemia?

Answer 7

Antibodies attack RBC

Warm

• haemolysis occurs at normal or warm temp
• often idiopathic, SLE, drugs e.g. methyldopa

Cold

• due to lymphoma, leukaemia, HIV, EBV, mycoplasma infection
• occurs at <10 degrees
• antibodies attach to RBC causing agglutination and breakdown

Treat with transfusions, prednisolone, rituximab and splenectomy if needed

Question 8

What is pyruvate kinase deficiency?

Answer 8

Autosomal recessive
Lack of ATP causing shortened RBC lifespan
If homozygous then will present with neonatal jaundice

Treat with folate and splenectomy if severe