Paediatric Haematology & Malignancy Flashcards
Complications of iron deficiency in childhood
Reduced cognitive and psychomotor performance (in the absence of anaemia)
CBE, iron studies and blood film in iron deficiency anaemia
Low haemoglobin Low MCV Low Ferritin Low serum iron Low transferring saturation High total iron binding capacity Slightly high platelets Microcytosis, hypochromia, poikilocytosis, anisocytosis, target cells (severe anaemia), pencil cells, sometimes nucleated RBCs
Thalassaemia minor diagnosis
Low MCV (much lower than degree of anaemia) Diagnosed on Hb electrophoresis (HbA2 greater than 3.5%) Pre-pregnancy carrier testing of partner is important
Macrocytic anaemia in children
Rare in children
Must be investigated and treated urgently if associated with FTT or neurodevelopmental problems
Symptoms and signs of anaemia in children
Lethargy Irritability Poor feeding Weakness Shortness of breath Pallor Changes in colour or urine, sclera, skin \+/- splenomegaly Flow murmur Signs of cardiac failure
Investigations to perform in microcytic anaemia
Iron studies:
- Low ferritin = iron deficiency
- High ferritin = sideroblastic or anaemia of chronic disease
- Normal ferritin - perform electrophoresis
Haemoglobin electrophoresis
HbA2 greater than 3.5%
Lead levels if at risk (chronic lead poisoning also leads to a microcytic anaemia picture)
Investigations to perform in undifferentiated anaemia
CBE, blood film and reticulocyte
Investigatinos to perform in normocytic anaemia
Reticulocytes:
- increased = haemolysis OR blood loss
Normal reticulocyte count OR abnormalities of other parameters
- hypoplastic/aplastic anaemias (marrow hypoplasias, leukaemia, infiltration)
Investigations for haemolytic anaemia
Blood film - ?RBC abnormalities e.g. spherocytosis Coomb's test G6PD screen Bilirubin Reticulocytes
Management of iron deficiency in children
Dietary modification
- optimise red meat, chicken, green vegetables, fortified foods
- limit cow’s milk to less than 500mL/day
Iron supplementation
Dosing and duration for iron supplementation in child
2-6mg/kg/day
Split into TDS to reduce gastric irritation
Continue for 3 months to replenish stores
Different mixtures of iron supplements for children
Ferrous sulphate
- better absorbed, less tolerated
- e.g. ferro-liquid (6mg.mL elemental iron)
Ferrous gluconate
Expected response to adequate iron supplementation in a child
Hb should rise by 10 each week
Follow up with reticulocyte response in 4 weeks
Prevention of iron deficiency anaemia in children
Introduction of iron containing solids at 4-6 months
Avoid cow’s milk for first 12 months - should only form very minor part of diet up to 24 months
Ensure all formulas and cereals are fortified
Consider supplementation in high risk groups (premmies, low birth weight)
Presentation of G6PD deficiency
Acute haemolysis: jaundice, pallor, dark urine
Acute anaemia: faitgue SOBOE or rest, confusion, lethargy
How severe must haemolysis be to cause anaemia
Greater than 5% of RBC mass per day
Screening and confirmatory tests for G6PD
Fluorescent spot test (most reliable and most sensitive - G6P and NADP to a haemolysate of test RBCs, measure NADPH after by direct fluorescence or nitro blue dye)
Confirmatory:
As above but measures amount of RBC haemolysate, measure NADPH production spectrophotometrically as units per gram of haemoglobin
Management of G6PD
Neonatally: manage as other kinds of neonatal jaundice
Acute presentation:
Haemolysis itself is self-limited
Future avoidance of drugs known to trigger haemolysis and fava beans
Transfusion more likely to be required in children and people with comorbidities causing impaired erythropoiesis
Diseases indicated by bleeding in joints
Haemophilia A and B
Disease processes indicated by mucosal bleeding
Local irritation
Von Willebrane disorder
Platelet dysfunction
Disease processes indicated by bleeding of gums, periosteum and skin
Scurvy
Disease processes indicated by gastrointestinal bleeding in paediatrics
Haemorrhagic disease of the newborn
Liver disease
What is haemorrhagic disease of the newborn?
Vitamin K deficient bleeding in a newborn who has not received a supplemental vitamin K injection
Disease processes indicated by retro-orbital bleeding in a child
Haematological malignancy
Disseminated solid tumour
Disease indicated by child bleeding from shins only
Not pathological on it’s own. Common in pre-schoolers or junior primary children. Ensure to exclude other sites of bleeding
Commonest cause of clotting disorders in children
Immune thrombocytopaenic purpura (ITP)
Epidemiology of ITP (incidence and common age)
4/100,000 per year
2-10 years, peaks at 5 years
Pathophysiology of ITP
Destruction of circulating platelets by anti-platelet IgG autoantibodies causing splenic sequestration
Presentation of ITP
Preceding viral infection 1-2 weeks before presentation
Petechiae, purpura and/or superficial bruising
Rarely causes profuse o mucosal bleeding
Bleeding usually occurs abruptly
Examination otherwise normal (nil lymphadenopathy or splenomegaly)
