Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Paediatrics and Child Health > Haematology and Oncology > Flashcards

Haematology and Oncology Flashcards

1
Q

How is childhood anaemia defined?

A

<11g/dL in children 6 months to 5 years

<11.5g/dL in children 5 to 11 years

<12g/dL in children 12 years and over

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is the most common cause of anaemia in children?

A

Iron deficiency anaemia - due to rapid growth and diet

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

In which ethnic groups is sickle cell disease seen more frequently?

A

Central Africa

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

In which ethnic groups is beta thalassaemia seen more frequently?

A

SE Asia
Mediterranean
Middle Eastern

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

How can the causes of anaemia be classified?

A
  1. Reduced Hb/RBC production
  2. Increased RBC destruction/haemolysis
  3. Blood loss
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Give three causes of anaemia due to reduced RBC/Hb production?

A 
Bone marrow aplasia
Bone marrow replacement by tumour cells
Bone marrow replacement by fibrous tissue/granulomas
Iron deficiency
B12 deficiency/pernicious anaemia
Folate deficiency
Thalassaemias
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Give two examples of bone marrow aplasia

A

Fanconi’s anaemia (congenital aplastic anaemia)

Diamond-blackfan anaemia (red blood cell aplasia)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Which conditions cause bone marrow replacement by:

(i) tumour cells?
(ii) fibrous tissue/granulomas?

A

i. Leukaemias, secondary mets

ii. TORCH infections

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Why might a child become deficient in:

(i) folic acid?
(ii) B12?

A

i. rapid growth, malabsorption (coeliac)

ii. breast feeding from vegetarian mother, malabsorption (coeliac)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What can cause anaemia due to haemolysis?

A

Genetic causes:
RBC membrane defects - hereditary spherocytosis
RBC enzyme abnormalities - G6PDD
Haemoglobinopathies - sickle cells disease, thalassaemias

Acquired causes:
Isoimmune haemolysis - HDoN, blood transfusion reactions
Infections, drugs and toxins

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What does the reticulocyte count show?

A

Low in iron deficiency

Increased in haemolysis/blood loss

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are the markers of iron deficiency?

A

Low reticulocytes
Low MCV
Low ferritin
Pale and small - HypOchromic and micrOcytic cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Who is iron deficiency more commonly seen in?

A

Preterm babies
Twins
Infants fed mainly on cows milk/late weaners
Social deprivation/poverty: Less red meat, green veg, eggs and bread
Chronic malabsorption (coeliac)
Adolescent girls - rapid growth, menstruation
Pica

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is pica?

A

When children eat almost anything, including soil or dirt

Latin name for magpie

Can cause lead poisoning if paint is ingested

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What signs might be apparent on clinical examination in a child with anaemia?

A 
Growth/FTT
Angular cheilosis
Koilonychia
Jaundice (haemolysis)
Clubbing (chronic illness)
Lymphadenopathy, hepatosplenomegaly, brusing (leukaemia)
Abdominal distension (coeliac)
Exertional tachycardia
Pale conjunctiva
SOB
Fatigue/Irritability
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What dysmorphic features might be apparent in a child with Fanconi’s anaema?

A 
Short stature
Microcephaly
Frontal bossing
Absent thumbs
Hyperpigmented skin
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

In what case of anaemia would jaundice be present?

A

Haemolysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What signs of anaemia might you see in a child with leukaemia?

A

Lymphadenopathy
Hepatosplenomegaly
Brusing

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What type of cells are seen in G6PDD?

A

Ghost or bite cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

When might you carry out red cell enzyme studies?

A

If suspecting G6PDD or pyruvate kinase deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Which anameia does Coombs test identify?

A

Autoimmune haemolytic anaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

How is iron deficiency anaemia managed?

A

Sytron (iron supplements) for 3 months

Transfusion (if severe)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

When would a child with anaemia require transfusion?

A

High-output cardiac failure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What mode of inheritance is hereditary spherocytosis?

A

Autosomal dominant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

What mode of inheritance is G6PDD?

A

X-linked recessive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Which type of anaemia does lead poisoning cause?

A

Microcytic anaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

What mode of inheritance is sickle cell disease?

A

Autosomal recessive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

In SCD, where does the mutation occur?

A

Beta globin gene

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

What does the presence of sickle cells cause disease?

A

They have a reduced deformability and are easily destroyed

This causes occlusion of microcirculation and a chronic haemolytic anaemia of about 90g/dL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

What are the different types of sickle cell disease?

A

HbSS/sickle cell anaemia - severe phenotype
HbS /beta0 thalassaemia -
severe, indistinguishable from HbSS
HbSC disease - HbS and HbC - intermediate severity
HbS/ beta+ thalassaemia - mild/moderate severity
HbS /hereditary persistence of fetal Hb - symptom free

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

What are the typical Hb proportions in sickle cell trait?

A

60% HbA and 40% HbS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

What does sickle cell trait protect against?

A

Malaria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Where is SCT most prevalent?

A

Sub-Saharan Africa

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

What are the clinical features of SCT?

A

Generally asymptomatic - often have no abnormal clinical findings

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Give three potential complications of SCT

A 
Haematuria
Decreased ability to concentrate urine
Renal papillary necrosis
Renal medullary cancer
Splenic infarction
Exertional rhabdomyolysis
Sudden death: induced by hypoxia, dehydration or exercise
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Which investigation is usually abnormal in SC trait?

A

Electrophoresis shows Hb AS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

How should you advise a parent of a child with SCT?

A

Keep them adequately hydrated

Avoid fluid loss and severe heat to prevent complications

38
Q

When do symptoms usually begin in children with SCD?

A

3 - 6 months of age - HbF levels begin to fall

39
Q

Why are kids with SCD more susceptible to infection?

A

Acute splenic sequestration

40
Q

How does a child with SCD clinically present?

A 
Anaemic
Jaundice
Pallor
Lethargy
Growth restirction/FTT
General weakness
Delayed puberty
Splenomegaly
41
Q

How does SCD usually cause anaemia?

A
  1. Acute splenic sequestration
  2. Transient red cell aplasia
  3. Hyperhaemolysis in patients with severe infection
42
Q

What are the different types of sickle cell crises?

A

Vaso-occlusive crisis
Aplastic crisis
Sequestration crisis
Acute chest syndrome

43
Q

What is an aplastic crisis?

A

Cessation of erythropoiesis cause severe anaemia

44
Q

What often precipitates an aplastic crisis?

A

Parvovirus B19 infection

45
Q

How might kids in aplastic crisis present?

A

Congestive cardiac failure

46
Q

What might kids in aplastic crisis need?

A

Transfusion

47
Q

What happens in a vaso-occlusive crisis?

A

There is obstruction of the microcirculation by sickle cells, causing ischaemia

48
Q

What usually precipitates a vaso-occlusive crisis?

A

Cold
Infection
Dehydration

49
Q

How might a child with a vaso-occlusive crisis present?

A

Pain - loin pain/abdominal pain/swollen painful joints
Tachypnoea
Fits and focal neurological signs
Retinal occlusion

50
Q

What can happen if the vaso-occlusive crisis involves large vessels?

A

Thrombotic stroke
Acute sickle chest syndrome
Placental infarction

51
Q

Why might a child in vaso-occlusive crisis get abdominal pain?

A

Mesenteric sickling

Bowel ischaemia

52
Q

Why might a child in vaso-occlusive crisis get loin pain?

Which other symptoms might accompany this?

A

Renal papillary necrosis

Haematuria

53
Q

What is a sequestration crisis?

A

Sequestration crises results from trapping of large amounts of red cells in the spleen and sometimes in the liver

54
Q

Who does a sequestration crisis more commonly occur in?

A

Babies and infants

55
Q

What is acute chest syndrome?

What is its clinical presentation?

A

Vaso-occlusive crisis affecting the lungs

Characterised by pleuritic chest pain, fever and new pulmonary infiltrate on CXR

56
Q

What is the most common crisis in kids with SCD?

A

Vaso-occlusive crisis

2nd most common reason for admission is acute chest syndrome

57
Q

How is a painful crisis usually managed?

A

Analgesia
Fluids
Warmth
Rest

Avoid cold exposure
Look for cause e.g. infection and treat

58
Q

Which drug can be used to reduce the frequency of crisis occurrence|?

A

Hydroxycarbamide

59
Q

When are children with SCD given prophylactic penicillin?

A

By 3 months of age

60
Q

Which infection are children with SCD more likely to get and specifically vaccinated against?

A

Pneumococcal

61
Q

What is the management of acute chest syndrome?

A

CPAP
Exchange transfusion
Antibiotics - macrolide + IV cephalosporin

62
Q

What investigation is done to monitor stroke in children with SCD?

A

Transcranial doppler USS

63
Q

What other potential complication in kids with SCD requires immediate management?

A

Priapism (sustained erection) - hydration, analgesia, adrenaline and etilefrine

64
Q

Give three other complications of SCD

A 
Priapism
Chronic pain
Nocturnal enuresis
Infection
Stroke
Cor pulmonale
Gallstones
Retinopathy
Chronic leg ulcers
Avascular necrosis of femoral/humeral head
CKD
Learning difficulties
65
Q

How is diagnosis of sickle cell crisis made?

A

Positive sickling test wit HbS on electrophoresis

66
Q

When would you urgently refer a child with SCD to hospital?

A 
Severe pain
Dehydration
Severe sepsis
Acute chest syndrome
Neurological signs
Symptoms/signs of acute fall in Hb
Acute enlargement of spleen
Jaundice
Haematuria
Priapism lasting more than 2 hours or worsening
67
Q

What mode of inheritance are thalassaemias?

A

Autosomal recessive

68
Q

What is the difference between thalassaemia minor and major?

A

Minor - heterozygous state - thalassaemia trait
Those with the trait are suually asymptomatic or have mild microcytic anaemia

Major - homozygous state - disease

69
Q

When does thalassaemia present?

A

Alpha: at birth there are symptoms of haemolytic anaemia (eg, pallor and hepatosplenomegaly)

Beta: usually in the latter part of the first year of life but can be as late as 5 years old because of delay in stopping HbF production

70
Q

How does thalassaemia usually appear on FBC?

A 
Microcytic hypochromic anaemia
Low Hb
Low MCV
Low MCH
Raised iron and ferritin
71
Q

Which other clinical signs can be seen in thalassaemia?

A 
Hepatosplenomegaly
Bone deformities e.g. frontal bossing, prominent facial bones, dental malocclusion
Marked pallor
Mild-moderate jaundice
Exercse intolerance
Cardiac flow murmur
HF secondary to anaemia
FTT/growth restriction
72
Q

What are the main complications of alpha thalassaemia?

A

Hydrops fetalis

Perinatal death

73
Q

What is done to prevent iron overload in thalassaemia patients?

A

Desferrioxamine bu subcutaneous infusion

74
Q

What is doen to support RBC production in thalassaemia patients?

A

Folic acid and Vit C supplements

Regular blood transfusions

75
Q

Give three complications of thalassaemia

A 
Iron overload (therefore endocrine dysfunction)
Transfusion risks
Osteoporosis
Gall stones/gout
Hepatocellular carcinoma
76
Q

What is the prognosis for severe beta thalassaemia?

A

80% die within five years

77
Q

What is HSP?

A

IgA mediated autoimmune hypersensitivity vasculitis

78
Q

Who is HSP more common in?

A

Female
Caucasians
4-6 years old

79
Q

Which four organs does HSP involve?

A

Skin
Joints
Gut
Kidneys

80
Q

What are the risk factors for HSP?

A

Infection: Group A strep, mycoplasma, EBV
Vaccinations
Environmental factors: allergens, pesticides, cold, insect bites

81
Q

How does HSP present?

A 
Winter months following URTI
Low grade fever
Purpuric rash - gravity-dependent
Abdominal pain/vomiting/bloody diarrhoea
Swollen tender knees/ankles
Renal injury - haematuria
Scrotal pain (mimics testicular torsion)
Headaches
Intussusception
82
Q

How does the HSP rash develop?

A

Symmetrical erythematous macular rash on back of legs, buttocks and ulnar side of arms

Evolves into raised purpuric lesions that may coalesce and resemble bruises

83
Q

How is HSP diagnosed?

What do investigations show?

A

Clinical diagnosis

Urinalysis: haem/proteinuria
Raised WCC with eosinophilia
Raised ESR
Raised Cr
Raised IgA
Autoantibody screen
Abdmonal US/braium enema for ?obstruction
Renal biopsy
84
Q

What are the renal complications of HSP?

A

Renal involvement is serious in 10%

ESKD (<1%)
Nephrotic syndrome
Oliguria
Hypertension

85
Q

How should HSP be managed?

A

HSP is self-limiting, so supportive

NSAIDs for joint pain, but not in renal insufficiency

Steroids/Azathioprine/Cyclophosphamide/Plasmapheresis in renal disease

Corticosteroids for arthralgia and intestinal dysfunction

Plasma exchange (for rapidly progressive nephritis)

86
Q

How should HSP patients with renal involvement be followed up?

A

If proteinuria, monthly

No proteinuria - regular blood pressure checking and urinalysis

87
Q

What are the other complications of HSP?

A 
MI
Pulmonary haemorrhage
Pleural effusion
Intussusception
GI bleed
Bowel infarction
Seizures
Mononeuropathies
88
Q

What is the prognosis for HSP?

A

Usually resolves but 1 in 4 will recur

The older you present, the worse the course and renal involvement

Younger than 3 years have a shoter, milder course with fewer recurrences

Prognosis largely depends on degree of renal involvement

89
Q

What is deficient in Haemophilia A and B?

A

A - Factor VIII

B - Factor IX

90
Q

Which haemophilia is:

  1. more common?
  2. more severe?
A

A

91
Q

What is the mode of inheritance of haemophilia?

A

X-linked recessive

Paediatrics and Child Health (11 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions