Paediatric Haematology & Malignancy Flashcards Preview

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Flashcards in Paediatric Haematology & Malignancy Deck (73)
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1

Complications of iron deficiency in childhood

Reduced cognitive and psychomotor performance (in the absence of anaemia)

2

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

3

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

4

Macrocytic anaemia in children

Rare in children
Must be investigated and treated urgently if associated with FTT or neurodevelopmental problems

5

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

6

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)

7

Investigations to perform in undifferentiated anaemia

CBE, blood film and reticulocyte

8

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)

9

Investigations for haemolytic anaemia

Blood film
- ?RBC abnormalities e.g. spherocytosis
Coomb's test
G6PD screen
Bilirubin
Reticulocytes

10

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

11

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

12

Different mixtures of iron supplements for children

Ferrous sulphate
- better absorbed, less tolerated
- e.g. ferro-liquid (6mg.mL elemental iron)
Ferrous gluconate

13

Expected response to adequate iron supplementation in a child

Hb should rise by 10 each week
Follow up with reticulocyte response in 4 weeks

14

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)

15

Presentation of G6PD deficiency

Acute haemolysis: jaundice, pallor, dark urine
Acute anaemia: faitgue SOBOE or rest, confusion, lethargy

16

How severe must haemolysis be to cause anaemia

Greater than 5% of RBC mass per day

17

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

18

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

19

Diseases indicated by bleeding in joints

Haemophilia A and B

20

Disease processes indicated by mucosal bleeding

Local irritation
Von Willebrane disorder
Platelet dysfunction

21

Disease processes indicated by bleeding of gums, periosteum and skin

Scurvy

22

Disease processes indicated by gastrointestinal bleeding in paediatrics

Haemorrhagic disease of the newborn
Liver disease

23

What is haemorrhagic disease of the newborn?

Vitamin K deficient bleeding in a newborn who has not received a supplemental vitamin K injection

24

Disease processes indicated by retro-orbital bleeding in a child

Haematological malignancy
Disseminated solid tumour

25

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

26

Commonest cause of clotting disorders in children

Immune thrombocytopaenic purpura (ITP)

27

Epidemiology of ITP (incidence and common age)

4/100,000 per year
2-10 years, peaks at 5 years

28

Pathophysiology of ITP

Destruction of circulating platelets by anti-platelet IgG autoantibodies causing splenic sequestration

29

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)

30

Diagnosis of ITP

Diagnosis of exclusion
CBE: platelets usually