Paediatric Haematology Flashcards

1
Q

What is Idiopathic Thrombocytopaenic Purpura?

A

Idiopathic Thrombocytopenic Purpura

Most common cause of thrombocytopenia in children

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2
Q

What is the cause of Idiopathic Thrombocytopaenic Purpura?

A

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

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3
Q

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

A

Recent viral infection or immunisation

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4
Q

What age is Idiopathic Thrombocytopaenic Purpura most common?

A

2-10 yo

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5
Q

How does Idiopathic Thrombocytopaenic Purpura present?

A

Commonly asymptomatic

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

Some have enlarged spleens

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6
Q

What differentials would you consider for Idiopathic Thrombocytopaenic Purpura?

A
Aplastic anaemia
Leukaemia
Von-Willebrand
Meningococcal septicaemia
HSP
Non Accidental Injury
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7
Q

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

A

FBC
Peripheral blood smear
Can do Bone Marrow Aspirate

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8
Q

What would an FBC show in Idiopathic Thrombocytopaenic Purpura?

A

Thrombocytopenia but no pancytopenia

Characteristic increase in megakaryocytes

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9
Q

How does Idiopathic Thrombocytopaenic Purpura normally progress?

A

Normally an acute, benign self limiting condition

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10
Q

What is Idiopathic Thrombocytopaenic Purpura treatment based on?

A

Symptoms

NOT PLATELET COUNT

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11
Q

What is the general management for Idiopathic Thrombocytopaenic Purpura?

A

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

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12
Q

When is pharmacological treatment suggested in Idiopathic Thrombocytopaenic Purpura?

A

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

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13
Q

What pharmacological management may be advised for Idiopathic Thrombocytopaenic Purpura?

A

1 Prednisolone
2 IV Immunoglobulin
3 Rituximab
4 High dose dexamethasone

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14
Q

What is the prognosis for Idiopathic Thrombocytopaenic Purpura?

A

80% completely recover within 6 months

Some children have chronic thrombocytopenia - higher risk in teenage girls

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15
Q

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

A

Splenectomy

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16
Q

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

A

<11g/dl

17
Q

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

A

<11.5g/dl

18
Q

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

A

<12g/dl

19
Q

How can anaemia be classified?

A

Colour - hypochromic/normochromic

Size - microcytic, normocytic, macrocytic

20
Q

What symptoms would anaemia’s present with?

A
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
21
Q

What cardiac changes could be seen in anaemia?

A

Exertional tachycardia
Gallop rhythm
Systolic flow murmur

22
Q

What is the most common cause of anaemia in children?

A

Iron deficiency anaemia

23
Q

What mechanisms can cause anaemia?

A

Decreased RBC or Hb production

Increased destruction

Blood loss

24
Q

What are the causes of microcytic anaemia?

A

Defects in haem or globin synthesis:

Iron deficiency
Chronic inflammation
Thalassaemia

25
Q

What are the causes of normocytic anaemia?

A

Haemolytic anaemias
Haemorrhage
Hypo-production

26
Q

What causes macrocytic anaemia?

A

Bone marrow megaloblastic:
Vitamin B12 deficiency
Folic acid deficiency

Bone marrow not megaloblastic:
Hypothyroidism
Fanconi anaemia

27
Q

What is fanconi’s anaemia?

A

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

28
Q

Give some causes of haemolytic anaemias

A

Red cell defects - spherocytosis, sickle cell, G6PD deficinecy

Extrinsic disorders - autoimmune mediated, hypersplenism, septic, DIC, haemolytic disease of the newborn

29
Q

What could cause B12 deficiency?

A

Breast fed by vegetarian and in malabsorption

30
Q

What could cause folate deficiency?

A

IBD
Coeliac
Anticonvulsants

31
Q

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

A

Marrow infiltration with malignant cells

Marrow replaced with fibrous tissue in TORCH infections

32
Q

What investigations would you request to investigate anaemia?

A
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
33
Q

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

A

Fetus absorb iron from mother across placenta

Term infants have adequate reserves for 4 months

Preterm run out by 8 weeks

34
Q

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

A

50% of iron absorbed from breast milk compared to 10% from cow’s

Formula milk has more iron but only 4% absorbed

35
Q

What can affect iron absorption in the diet?

A

Enhanced by vit C

Reduced by tannin (in tea)

36
Q

When does iron requirement increase for children?

A

Adolescence - esp girls who lose iron through menstruation

37
Q

Give some causes of iron deficiency

A
Menstruation
Hookwork infestation
Repeated venesection in babies
Meckel's diverticulum
Recurrent epistaxis
38
Q

How is iron deficiency managed?

A

Avoid unmodified cow’s milk
Iron supplements - sodium feredetate

In severe cases, may need transfusion