Paediatric Anaemia Flashcards
what are the causes of iron deficiency anaemia
diet tropical sprue coeliac disease crohns disease GI blood loss
what is the treatment for iron deficiency anaemia in children
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
what does microcytic anaemia with high/normal ferritin suggest
thalassaemia
what type of microcytic anaemia do thalassaemias cause
hypochromic
what will you get if you have no working alpha chains
Hb barts hydrops foetalis
what happens if you cant make any beta chains
beta thalassaemia major; around 6 months there is a switch from gamma to beta (HbF to HbA) and this will result in anaemia
what is high HbA2 diagnostic of
beta thalassaemia major (need regular transfusions)
what Ix for thalassaemias
- 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
what is the treatment for thalassaemias
- Blood transfusion
- Prevent: extramedullary haematopoesis
what are the complications of extra meduallary haemstopoesis
hepatosplenomegaly, bony changes due to marrow expansion, frontal bossing, long bone malformations, spinal cord compression
what are the potential side effects of blood transfusion as tx for thalassaemia
- iron overload
- Liver cirrhosis
- Endocrine problems; diabetes, hypothyroidism, hypoparathyroidism
- Arthralgia
- Main cause of death in transfusion dependent thalassaemia patients is cardiomyopathy and arrhythmias
how do you prevent iron overload in repeated transfusions
iron chelate (slow accumulation of iron) via deferoxamine
why do patients with frequent transfusions have to have very specific blood matching done
as at high risk of antibody formation
how do you monitor tx efficacy in beta thalassaemia major
- Behaviour; meeting milestones, growth, development
- Look at Hb; should increase after transfusion
what tests picks up thalassaemias at birth
guthrie heel prick
Normocytic anaemia, thrombocytosis, hyperbilirubinemia = ?
haemolysis is occurring
what is a spherocyte
spherical red cell
what can cause spherocytes
- 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
why do you have raised reticulocytes in spherocytosis
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.
what happens when people with spherocytosis become ill with intercurrent illness
will become anaemic and will get jaundiced and may require transfusion
what happens to the spleen in spherocytosis
get splenomegaly as its removing so many of the spherocytes- why poeple with hereditary spherocytosis get anaemia
what are people with hyposplenism at risk of
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
what is seen in a haemolysis screen
- 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)
what tests show is someone has antibodies on their red cells
- Cross match – indirect coombs test (plasma from patient with donor red cells)
- Direct coombs/ antiglobulin test – red cells from patient and mixing with antibodies.
will coombs test be +ve in heriditary spehroctosis
no as no antibodies on red cells
will coombs test be +ve autoimmune haemolytic anaemia
yes
what are the causes of acquired haemolysis
autoimmune haemolysis (warm or cold) malaria
what is autoimmune haemolytic anaemia and its types
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
what Ix for hereditary spherocytosis
FHx spherocytes on blood film no agglutination on direct coombs testing genetic testing haemoglobin electrophoresis
what is the treatment for hereditary spherocytosis
folic acid (iron and B12 recycled)
splenectomy NOT indicated
transfusions only when unwell
what is haemoglobinuria
free Hb in urine (red cells already lyzed)
haematuria= red cells in urine
what should you think when someone with hereditary spherocytosis becomes suddenly anaemic
parovirus B19
what does parovirus B19 do to blood
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)
