Paediatric Anaemia

Question 1

what are the causes of iron deficiency anaemia

Answer 1

diet 
tropical sprue 
coeliac disease 
crohns disease 
GI blood loss

Question 2

what is the treatment for iron deficiency anaemia in children

Answer 2

iron supplementation orally (liquid suspension) and dietary advice. Would struggle to correct an iron deficiency anaemia with diet alone.
iron 3x a day (SE= constipation, black poo, nauseated)

Monitoring; check reticulocyte count to monitor effectiveness of iron treatment. Check 10 days after start of treatment. Expect an increase of 10g/L every 2 weeks

Question 3

what does microcytic anaemia with high/normal ferritin suggest

Answer 3

thalassaemia

Question 4

what type of microcytic anaemia do thalassaemias cause

Answer 4

hypochromic

Question 5

what will you get if you have no working alpha chains

Answer 5

Hb barts hydrops foetalis

Question 6

what happens if you cant make any beta chains

Answer 6

beta thalassaemia major; around 6 months there is a switch from gamma to beta (HbF to HbA) and this will result in anaemia

Question 7

what is high HbA2 diagnostic of

Answer 7

beta thalassaemia major (need regular transfusions)

Question 8

what Ix for thalassaemias

Answer 8

• Electrophoresis is the gold standard to look for HbA2
• HPLC will identify the Hb present – can identify HbF, HbA, HbA2, HbS. Not helpful for alpha thal (you will just see less of all Hb chains)
• To confirm alpha thal; you need to do genetic testing
• Beta thalassaemia; increased numbers of HbA2

Question 9

what is the treatment for thalassaemias

Answer 9

• Blood transfusion

- Prevent: extramedullary haematopoesis

Question 10

what are the complications of extra meduallary haemstopoesis

Answer 10

hepatosplenomegaly, bony changes due to marrow expansion, frontal bossing, long bone malformations, spinal cord compression

Question 11

what are the potential side effects of blood transfusion as tx for thalassaemia

Answer 11

• iron overload
• Liver cirrhosis
• Endocrine problems; diabetes, hypothyroidism, hypoparathyroidism
• Arthralgia
• Main cause of death in transfusion dependent thalassaemia patients is cardiomyopathy and arrhythmias

Question 12

how do you prevent iron overload in repeated transfusions

Answer 12

iron chelate (slow accumulation of iron) via deferoxamine

Question 13

why do patients with frequent transfusions have to have very specific blood matching done

Answer 13

as at high risk of antibody formation

Question 14

how do you monitor tx efficacy in beta thalassaemia major

Answer 14

• Behaviour; meeting milestones, growth, development

- Look at Hb; should increase after transfusion

Question 15

what tests picks up thalassaemias at birth

Answer 15

guthrie heel prick

Question 16

Normocytic anaemia, thrombocytosis, hyperbilirubinemia = ?

Answer 16

haemolysis is occurring

Question 17

what is a spherocyte

Answer 17

spherical red cell

Question 18

what can cause spherocytes

Answer 18

• Hereditary spherocytosis
• autoimmune haemolytic anaemia= Antibody attachment to the red cells that means when they pass through the spleen a little bit gets taken off resulting in a spherocyte

Question 19

why do you have raised reticulocytes in spherocytosis

Answer 19

Spherocytes will not live as long and will lyze quicker and therefore there is a compensatory increase in the number of red cells – increased reticulocyte count.

Question 20

what happens when people with spherocytosis become ill with intercurrent illness

Answer 20

will become anaemic and will get jaundiced and may require transfusion

Question 21

what happens to the spleen in spherocytosis

Answer 21

get splenomegaly as its removing so many of the spherocytes- why poeple with hereditary spherocytosis get anaemia

Question 22

what are people with hyposplenism at risk of

Answer 22

infections with encapsulated organisms; haemophilus, pneumococcus, meningococcus.

There is also an increased risk of DVT/ PE.

at risk of folate deficiency because of high turnover rate so put on folci acid

Question 23

what is seen in a haemolysis screen

Answer 23

• Retic count: increased
• LDH levels: increased
• Haptoglobin levels: decreased

LDH levels =marker of cell turnover
haptoglobin levels (chemical that lives in blood and is present in case red cells burst and will mob up Hb because free Hb is bad for the kidneys, will be decreased in haemolysis as being used up)

Question 24

what tests show is someone has antibodies on their red cells

Answer 24

• Cross match – indirect coombs test (plasma from patient with donor red cells)
• Direct coombs/ antiglobulin test – red cells from patient and mixing with antibodies.

Question 25

will coombs test be +ve in heriditary spehroctosis

Answer 25

no as no antibodies on red cells

Question 26

will coombs test be +ve autoimmune haemolytic anaemia

Answer 26

yes

Question 27

what are the causes of acquired haemolysis

Answer 27

autoimmune haemolysis (warm or cold) 
malaria

Question 28

what is autoimmune haemolytic anaemia and its types

Answer 28

Autoimmune haemolytic anaemia is a relatively rare condition caused by autoantibodies directed against a person’s own red blood cells. The condition has warm and cold antibody types.

Warm antibody type can be idiopathic or secondary to other conditions such as systemic lupus erythematosus, lymphoma, chronic lymphocytic leukaemia or Evans syndrome.

Cold antibody types include cold haemagglutinin disease and paroxysmal cold haemoglobinuria

Question 29

what Ix for hereditary spherocytosis

Answer 29

FHx
spherocytes on blood film
no agglutination on direct coombs testing 
genetic testing 
haemoglobin electrophoresis

Question 30

what is the treatment for hereditary spherocytosis

Answer 30

folic acid (iron and B12 recycled)
splenectomy NOT indicated
transfusions only when unwell

Question 31

what is haemoglobinuria

Answer 31

free Hb in urine (red cells already lyzed)

haematuria= red cells in urine

Question 32

what should you think when someone with hereditary spherocytosis becomes suddenly anaemic

Answer 32

parovirus B19

Question 33

what does parovirus B19 do to blood

Answer 33

causes an aplastic crisis (not producing sufficient rbcs)
in people with HS they are unable to cope with this (in normal people they can cope if their bone marrow stops producing red cells for a couple of days)