Paediatric Haematology

Question 1

What is Idiopathic Thrombocytopaenic Purpura?

Answer 1

Idiopathic Thrombocytopenic Purpura

Most common cause of thrombocytopenia in children

Question 2

What is the cause of Idiopathic Thrombocytopaenic Purpura?

Answer 2

Autoimmune disorder causing destruction of platelets leading to reduced circulating platelet numbers

Question 3

What is common in the history of children with Idiopathic Thrombocytopaenic Purpura?

Answer 3

Recent viral infection or immunisation

Question 4

What age is Idiopathic Thrombocytopaenic Purpura most common?

Answer 4

2-10 yo

Question 5

How does Idiopathic Thrombocytopaenic Purpura present?

Answer 5

Commonly asymptomatic

Can see:
Purpura and superficial bleeding
Petechiae
Nosebleeds
Intracranial haemorrhage
Menorrhagia
GI bleeds

Some have enlarged spleens

Question 6

What differentials would you consider for Idiopathic Thrombocytopaenic Purpura?

Answer 6

Aplastic anaemia
Leukaemia
Von-Willebrand
Meningococcal septicaemia
HSP
Non Accidental Injury

Question 7

What investigations would you request if you suspect Idiopathic Thrombocytopaenic Purpura?

Answer 7

FBC
Peripheral blood smear
Can do Bone Marrow Aspirate

Question 8

What would an FBC show in Idiopathic Thrombocytopaenic Purpura?

Answer 8

Thrombocytopenia but no pancytopenia

Characteristic increase in megakaryocytes

Question 9

How does Idiopathic Thrombocytopaenic Purpura normally progress?

Answer 9

Normally an acute, benign self limiting condition

Question 10

What is Idiopathic Thrombocytopaenic Purpura treatment based on?

Answer 10

Symptoms

NOT PLATELET COUNT

Question 11

What is the general management for Idiopathic Thrombocytopaenic Purpura?

Answer 11

No contact sports
Avoid NSAID’s
Look out for red flags

Question 12

When is pharmacological treatment suggested in Idiopathic Thrombocytopaenic Purpura?

Answer 12

Life-threatening bleeding
Increased risk of bleeding - haemophilia
Prior invasive procedure

Question 13

What pharmacological management may be advised for Idiopathic Thrombocytopaenic Purpura?

Answer 13

1 Prednisolone
2 IV Immunoglobulin
3 Rituximab
4 High dose dexamethasone

Question 14

What is the prognosis for Idiopathic Thrombocytopaenic Purpura?

Answer 14

80% completely recover within 6 months

Some children have chronic thrombocytopenia - higher risk in teenage girls

Question 15

What is used for Idiopathic Thrombocytopaenic Purpura if medical therapies fail?

Answer 15

Splenectomy

Question 16

What Hb level signifies anaemia in a child <5yo?

Answer 16

<11g/dl

Question 17

What Hb level signifies anaemia in a child between 5-11yo?

Answer 17

<11.5g/dl

Question 18

What Hb level signifies anaemia in a child between 12-14yo?

Answer 18

<12g/dl

Question 19

How can anaemia be classified?

Answer 19

Colour - hypochromic/normochromic

Size - microcytic, normocytic, macrocytic

Question 20

What symptoms would anaemia’s present with?

Answer 20

Fail to thrive
Fatigue
Short of breath
Cardiac changes
Growth affected but head circumference preserved
Pallor
Petechiae or bruising
Splenomegaly - haemolysis?
Jaundice
Small stature, small head and frontal bossing

Question 21

What cardiac changes could be seen in anaemia?

Answer 21

Exertional tachycardia
Gallop rhythm
Systolic flow murmur

Question 22

What is the most common cause of anaemia in children?

Answer 22

Iron deficiency anaemia

Question 23

What mechanisms can cause anaemia?

Answer 23

Decreased RBC or Hb production

Increased destruction

Blood loss

Question 24

What are the causes of microcytic anaemia?

Answer 24

Defects in haem or globin synthesis:

Iron deficiency
Chronic inflammation
Thalassaemia

Question 25

What are the causes of normocytic anaemia?

Answer 25

Haemolytic anaemias Haemorrhage Hypo-production

Question 26

What causes macrocytic anaemia?

Answer 26

Bone marrow megaloblastic: Vitamin B12 deficiency Folic acid deficiency Bone marrow not megaloblastic: Hypothyroidism Fanconi anaemia

Question 27

What is fanconi's anaemia?

Answer 27

Congenital aplastic anaemia - decreased bone marrow production of all blood cells

Question 28

Give some causes of haemolytic anaemias

Answer 28

Red cell defects - spherocytosis, sickle cell, G6PD deficinecy Extrinsic disorders - autoimmune mediated, hypersplenism, septic, DIC, haemolytic disease of the newborn

Question 29

What could cause B12 deficiency?

Answer 29

Breast fed by vegetarian and in malabsorption

Question 30

What could cause folate deficiency?

Answer 30

IBD Coeliac Anticonvulsants

Question 31

What are some other causes for decreased RBC or Hb production?

Answer 31

Marrow infiltration with malignant cells | Marrow replaced with fibrous tissue in TORCH infections

Question 32

What investigations would you request to investigate anaemia?

Answer 32

``` FBC and MCV B12 and folate Ferritin - iron deficiency Reticulocyte count Blood film Red cell enzyme studies Coomb's test - autoimmune haemolysis Endomysial antibodies - coeliac's ```

Question 33

Why are pre-term infants at risk of Iron Deficiency anaemia?

Answer 33

Fetus absorb iron from mother across placenta Term infants have adequate reserves for 4 months Preterm run out by 8 weeks

Question 34

Why is type of milk important when considering iron deficiency anaemia?

Answer 34

50% of iron absorbed from breast milk compared to 10% from cow's Formula milk has more iron but only 4% absorbed

Question 35

What can affect iron absorption in the diet?

Answer 35

Enhanced by vit C Reduced by tannin (in tea)

Question 36

When does iron requirement increase for children?

Answer 36

Adolescence - esp girls who lose iron through menstruation

Question 37

Give some causes of iron deficiency

Answer 37

``` Menstruation Hookwork infestation Repeated venesection in babies Meckel's diverticulum Recurrent epistaxis ```

Question 38

How is iron deficiency managed?

Answer 38

Avoid unmodified cow's milk Iron supplements - sodium feredetate In severe cases, may need transfusion