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Year 4 PAEDS > Haematology and Oncology > Flashcards

Haematology and Oncology Flashcards

1
Q

What is the most common type of anaemia?

A

Iron deficiency anaemia (IDA)

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2
Q

List some risk factors for IDA

A

Pregnancy:

  • Preterm
  • LBW infants
  • Multiple births

Nutrition:

  • After exclusive breast feeding for >6mnths
  • Delayed introduction of iron-containing solids
  • Children fed mostly cows milk (low in iron)

Adolescent females:

  • Growth spurt
  • Menstruation

Low iron in diet:

  • Poverty
  • Veganism

Malabsorption:

  • Coeliac disuse
  • IBD
Blood loss (rare): 
(NB 1ml blood loss = 0.5mg iron)
- Mickel's diverticulum
- Oesophagitis
- Cysts
- Tumours
- NSAIDs

Intestinal parasites:
- Hookworms

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3
Q

What is pica?

A

Associated with IDA

A disorder characterised by appetite for non-nutritive substances eg ice, hair, paper, stones, soil etc

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4
Q

How should IDA be prevented in the newborn?

A

Newborns have approx 4 months of iron stores

From 4 months exogenous iron should be given of 1mg/kg/day (if exclusively breastfed)

From 6 months should be started on iron containing foods

Avoid non-modified cows milk

Supplemented formula milk given to 24 months in high risk groups

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5
Q

How may IDA present in a child?

A

Most cases are subclinical. Symptoms only develop in severe IDA

1) Fatigue
2) Irritability
3) Failure to thrive
4) Exertional dyspnoea
5) Anaemic signs eg pallor, koilonychia
6) Jaundice (if haemolysis)
7) Reduced cognitive and psychomotor performance
8) Pica (rare)

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6
Q

What investigations can be done for IDA and what do they show?

A

1) FBC:
- Hb <11g/dL
- Decreased MCV
- Increased platelets

2) Blood film
- Hypochromic, microcytic RBCs

3) Serum ferritin
- Decreased = 99% positive predictive value

4) Serum iron
- Low in IDA
- Normal in thalassaemia

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7
Q

What are some ddx for hypochromic, microcytic anaemia? (5)

A

Microcytic anaemia = MCV <80fL

From insufficient haemoglobin production

Defective haem synthesis:

1) Iron deficiency anaemia (most common)
2) Lead poisoning
3) Anaemia of chronic disease (late phase)
4) Sideroblastic anaemia

Defective globin chain:
5) Thalassemia

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8
Q

What can cause normocytic anaemia? (7)

A

Normocytic anaemia = MCV 80-100 fL

Decreased volume and/or decreased erythropoiesis

Haemoglobinopathies:

1) Sickle cell anaemia
2) Haemoglobin C disease

Enzyme deficiencies:

3) Pyruvate kinase deficiency
4) G6PD deficiency

5) Blood loss
6) Aplastic anaemia
7) Anaemia of CKD

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9
Q

What can cause macrocytic anaemia?

A

Macrocytic anaemia = MCV >100fL

Insufficient cell production and / or maturation

Megaloblastic:

1) Vit B12 def
2) Folate def
3) Medications:
- Phenytoin
- Sulfa drugs
- Trimethoprim
- Hydroxyurea
- Methotrexate

Non-megaloblastic:

4) Liver disease
5) Alcohol use
6) Myelodysplastic syndrome
7) Hypothyroidism
8) Multiple myeloma

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10
Q

What is the treatment of IDA in children?

A

Diet correction: 2mg/kg/day oral ferrous salt, continue until 3 months after normal Hb

NB failure to respond to iron supplements = malabsorption

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11
Q

What is the most common site for solid tumours in childhood?

A

Brain

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12
Q

What structures may be involved in brain tumours?

A

Intracranial tumour affecting brain, meninges, pituitary gland, pineal gland and/or cranial nerves

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13
Q

Where are brain tumours in children most commonly?

A

Most (70-80%) = infratentorial
Tentorium separates the cerebrum (above) from the cerebellum (below)

ie most are in the cerebellum

eg Glial tumours or medulloblastomas

Also midline = germ cell tumours, craniopharyngiomas

(unlike adults where most are secondary and supratentorial)

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14
Q

What proportion of childhood cancers are brain and CNS?

A

One quarter

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15
Q

How are brain tumours classified?

A

Low-grade (1 and 2)

High-grade (3 and 4)

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16
Q

What does low-grade mean?

A

Grade 1 = doesn’t spread
Grade 2 sometimes spreads slowly and may recur if removed

Sometimes called ‘benign brain tumours’ but misleading as their mass effects mean they can still be harmful

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17
Q

What does high-grade mean?

A

Malignant tumours which spread to other tissues and tend to recur if removed

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18
Q

How do brain tumours typically present? What % are diagnosed in ED?

A

Progressive and subacute

60% diagnosed in ED

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19
Q

Give some examples of low-grade brain tumours?

A

1) Meningioma
2) Pituitary adenoma
3) Acoustic neuroma
4) Craniopharyngioma

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20
Q

What is an acoustic neuroma?

A

Benign tumour of CN8 (vestibulocochlear) Schwann cells

aka vestibular schwannoma

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21
Q

What are acoustic neuromas associated with?

A

Neurofibromatosis type 2

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22
Q

How may an acoustic neuroma present?

A

Gradual onset of unilateral sensorineural deafness and pressure in one ear

Followed by unilateral face numbness and absent corneal reflex (due to CN5 compression), vertigo, n&v

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23
Q

How are acrostic neuromas managed?

A

Slow growing so can be monitored with annual MRI

Removed with surgery or Gamma knife radiosurgery

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24
Q

What ages does craniopharyngiomas affect?

A

5-15 and 65-75yrs

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25
Q

Where do craniopharyngiomas arise? Where may they also affect?

A

Pituitary stalk

May affect hypothalamus and pituitary

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26
Q

How may a craniopharyngioma present?

A

Signs of raised ICP
Bitemporal hemianopia
Hydrocephalus
Endocrine symptoms

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27
Q

List some examples of high-grade brain tumours?

A

1) Gliomas
2) Medulloblastomas
3) Primary CNS lymphoma

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28
Q

What is the most common type of glioma?

A

Astrocytoma

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29
Q

What are types of astrocytoma?

A

Glioblastoma aka glioblastoma multiforme (GBM)
(most common malignant tumour in adults, life expectancy 1yr)

Pilocytic astrocytoma (PCA) = commonest benign tumours in kids → 5yr survival 95%

30
Q

Other than astrocystoma, what are other types of gliomas?

A

Ependymomas

Oligodendrogliomas

31
Q

What are medulloblastomas?

A

Tumour of cerebellar neurons

A type of primitive neuroectodermal tumour (PNET)

32
Q

How common are medulloblastomas?

A

Most common malignant tumours in children

33
Q

How may a medulloblastoma present?

What is the 5yr survival?

A

Hydrocephalus
Cerebellar signs eg abnormal gait and coordination

5yr survival is 70%

34
Q

What are primary CNS lymphomas most commonly?

A

Diffuse large B-cell lymphoma

35
Q

Which population are more likely to have primary CNS lymphomas?

A

Those with HIV

36
Q

What are the most common type of brain tumours in children?

A

Gliomas, either:

1) Astrocytomas (40%)
- very variable prognosis

2) Medulloblastomas (20%)
- most have spinal mets at diagnosis

38
Q

How may brain tumours present in children?

A

Mostly cerebellar signs = DANISH

Signs of raised ICP

+/- focal neurological signs depending on site of tumour
- eg squint from 6th nerve palsy, seizures, cerebellar signs, personality changes

40
Q

What is are cerebellar signs - DANISH?

A 
Dysdiadochokinesia
Ataxia
Nystagmus
Intension tremor
Scanning dysarthria
Heel-shin test +ve
42
Q

What are signs of raised ICP?

A 
Papilloedema
Altered LOC
Headache
Vomiting
Behavioural changes
Bulging fontanelle
Raised BP
Decreased HR
44
Q

How should brain tumours in children be investigated?

A

MRI with contrast

Never perform a LP if raised ICP

44
Q

Why should a LP never be performed if suspected raised ICP?

A

High pressure gradient from within cranium can cause herniation of brainstem through foramen magnum

44
Q

What should you do with a child presenting with persistent back pain?

A

Always do MRI with contrast

44
Q

What is the management of brain tumours?

A

Surgery = first line

Not all operable eg brainstem not

Chemo/radiotherapy

44
Q

What has the best survival rates of brain tumours?

A

Astrocytomas

45
Q

What is haemophilia?

A

Bleeding disorder caused by a deficiency of clotting factors

No fibrin is formed in blood clots leading to easy clot breakdown

46
Q

What are the two types of haemophilia?

A

Haemophilia A = Factor VIII deficiency
Haemophilia B = Factor IX deficiency

A8 B9

47
Q

What is the mode of inheritance of haemophilia?

A

X-linked recessive

Affects males born to carrier mothers

48
Q

How is severity of haemophilia A classified? What ages does each present?

A

% of FVIII activity

1) Mild
- >5% FVIII activity
- Presents >2yrs of age

2) Moderate
- 1-5% FVIII activity
- Presents <2yrs

3) Severe (most)
- <1% FVIII activity
- Presents in infancy

49
Q

What mutation causes haemophilia A?

A 
Usually FH (2/3rd)
But sporadic cases can occur due to novel mutations (1/3rd)
50
Q

What is more common haemophilia A or B?

A

A is 5x more common than B

51
Q

How may severe haemophilia A present?

A

1) Neonatal bleeding (1/3rd) eg following circumcision
2) Neonatal haemorrhage
3) Haematoma
4) Prolonged bleeding from cord or umbilical area
5) Hx of spontaneous bleeding into joints esp knees, ankles and elbows, without hx of significant trauma
6) IM haemorrhage
7) GI / mucosal haemorrhage - but more often haemophilia B/von Willibrand’s disease
8) Haematuria

52
Q

How may moderate haemophilia A present?

A

Bleeding after venepuncture

53
Q

How may mild haemophilia A present?

A

Bleeding after major trauma

54
Q

What may untreated cases of severe haemophilia A lead to? (4)

A

1) Athropothy and joint deformity
- May require replacement of affected joints

2) Soft tissue haemorrhages
- Can lead to compartment syndrome and neurological damage

3) Extensive retroperitoneal bleeds
- Can cause haemodynamic compromise

4) Haematoma formation
- May require fasciotomy

55
Q

List some ddx for haemophilia A (7)

A

1) Haemophilia B
2) Von Willebrand’s disease
3) Vit K deficiency / antagonism with antiogoagulants
4) Haemophilia C (factor XI deficiency)
5) Disorders of fibrinogen or fibrinolytic production
6) Platelet disorders
7) Blood vessel disorders

56
Q

What investigations are performed for haemophilia A?

A

FBC
- Low haematocrit and reduced Hb if recent bleeding

PT, bleeding time, fibrinogen levels and VWF
- Normal

APTT

  • Usually prolonged but can be normal if mild
  • Mixed pt plasma 1:1 with donor plasma should normalise

Factor VIII:C

  • Reduced
  • % activity shows severity of disease

Imagine arthtopathies with MRI or US

CT head if haemorrhage

57
Q

What is the prophylactic management of haemophilia A?

A

Infusions of factor VIII minimum once weekly

58
Q

What is a complication of haemophilia A?

A

Degenerative joint disease due to recurrent haemarthrosis

59
Q

What tends to be more severe - haemophilia A or B?

A

A = more severe

60
Q

What does the activated partial thromboplastin time (aPTT) measure?

A

The intrinsic and common pathways

61
Q

What is the intrinsic pathway?

A

HMWK (high molecular weight kininogen) activates:
FXII - XIIa

XIIa activates:
FXI to FXIa

FXIa activates:
FIX to FIXa

12 + 11 + 9

FVIIIa (8 = Haemophilia A) then with FIXa activates FX

62
Q

What does prothrombin time (PT) measure?

A

Extrinsic and common pathways

63
Q

What is the extrinsic pathway?

A

Tissue factor activates factor FVII - FVIIa

7 = heaven = extrinsic

64
Q

What is the common pathway?

A

Factor X is activated by FIXa (intrinsic) and FVIIa (extrinsic)

Xa AND Va then activate:
Prothrombin (FII) to thrombin (FIIa)

Thrombin activates:
Fibrinogen (FI) to Fibrin (FIa)

10 + 5 + thrombin + fibrin

65
Q

What is protein C?

A

= autoprothrombin IIa

A proenzyme that inactivates FVa and VIIIa

Too little = clot too much

66
Q

What % of haemophilia B are mild, moderate and severe?

A

Severe - 50%
Moderate - 3%
Mild - 20%

67
Q

In general, deficiencies in what cause:

a) Petechial haemorrhages and ecchymoses (bruising)
b) Haematomas and haemarthroses

A

a) Petechial haemorrhages and ecchymoses (bruising)
- platelet deficiency

b) Haematomas and haemarthroses
- Clotting factor deficiency

68
Q

What did haemophilia B used to be known as?

A

Christmas disease

69
Q

What are signs/symptoms of haemophilia B?

A

Same as A

May be slower to bleed

70
Q

What investigations are done for haemophilia B?

A

aPTT - elevated

% activity of FIX = reduced

71
Q

What is the management of haemophilia B?

A

Recombinant FIX usually twice a week

Year 4 PAEDS (23 decks)

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