Paediatric Anaemia

Question 1

What are the common causes for an iron deficiency?

Answer 1

Dietary deficiency
Tropical sprue
Coeliac disease 
Crohn's disease 
GI blood loss

Question 2

What is the first thing you should think about in a patient with microcytic anaemia but a normal ferritin count?

Answer 2

Thalassaemia

Question 3

What is the gold standard for diagnosis of thalassaemia?

Answer 3

Electrophoresis or HPLC (identifies Hb present; HbA2 high in beta thal)

Question 4

What is the purpose of blood transfusion in beta thal major?

Answer 4

Treat anaemia

Prevent extramedullary haematopoesis

Question 5

Are thalassaemias picked up at birth in the UK?

Answer 5

Yes; part of gurthrie screening

Question 6

What should you suspect in a patient with normocytic anaemia and hyperbilirubinaemia?

Answer 6

Haemolysis

Question 7

What is a spherocyte?

Answer 7

Spherical red cell

Question 8

What is hereditary spherocytosis?

Answer 8

Genetic condition that results in spherocytes in place of the usual biconcave RBC
Increased retic count

Question 9

What will happen when people with spherocytosis become unwell?

Answer 9

Struggle to compensate; will get anaemic and jaundice

May require transfusion

Question 10

Will you get organomegaly in spherocytosis?

Answer 10

Yes; splenomegaly

Used to treat HS with splenectomy but not done now as risks outweigh benefits

Question 11

What infections are people with hyposplenism more susceptible to?

Answer 11

Encapsulated organisms; haemophilus, pneumococcus, meningococcus

Question 12

What is the treatment for HS?

Answer 12

Folic acid
Transfusions if anaemic
?Splenectomy

Question 13

What is a haemolysis screen?

Answer 13

Reticulocyte count - marker of bone marrow activity
LDH levels - marker of cell turnover
Haptoglobin levels - mops up free Hb from lyzed red cells

Question 14

Should you do any antibody tests if someone has haemolysis?

Answer 14

Yes; need to rule out autoimmune haemolysis
Cross match/ indirect coombs test = test plasma from patient with donor red cells (looking for antibodies)
Direct coombs test = test red cells from patient and mix with plasma (looking for antigens present on the red cells)

Question 15

What can cause sudden anaemia in patients with spherocytosis?

Answer 15

Infection with parovirus B19 (slapped cheek) = results in aplastic crisis
In most people this is tolerated but in HS they will get very suddenly anaemic and jaundiced