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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

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2
Q

What is the most common cause of anaemia in children?

A

Iron deficiency anaemia - due to rapid growth and diet

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3
Q

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

A

Central Africa

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4
Q

In which ethnic groups is beta thalassaemia seen more frequently?

A

SE Asia
Mediterranean
Middle Eastern

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5
Q

How can the causes of anaemia be classified?

A
  1. Reduced Hb/RBC production
  2. Increased RBC destruction/haemolysis
  3. Blood loss
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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
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7
Q

Give two examples of bone marrow aplasia

A

Fanconi’s anaemia (congenital aplastic anaemia)

Diamond-blackfan anaemia (red blood cell aplasia)

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8
Q

Which conditions cause bone marrow replacement by:

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

A

i. Leukaemias, secondary mets

ii. TORCH infections

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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)

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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

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11
Q

What does the reticulocyte count show?

A

Low in iron deficiency

Increased in haemolysis/blood loss

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12
Q

What are the markers of iron deficiency?

A

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

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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

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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

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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
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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
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17
Q

In what case of anaemia would jaundice be present?

A

Haemolysis

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18
Q

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

A

Lymphadenopathy
Hepatosplenomegaly
Brusing

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19
Q

What type of cells are seen in G6PDD?

A

Ghost or bite cells

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20
Q

When might you carry out red cell enzyme studies?

A

If suspecting G6PDD or pyruvate kinase deficiency

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21
Q

Which anameia does Coombs test identify?

A

Autoimmune haemolytic anaemia

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22
Q

How is iron deficiency anaemia managed?

A

Sytron (iron supplements) for 3 months

Transfusion (if severe)

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23
Q

When would a child with anaemia require transfusion?

A

High-output cardiac failure

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24
Q

What mode of inheritance is hereditary spherocytosis?

A

Autosomal dominant

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25
What mode of inheritance is G6PDD?
X-linked recessive
26
Which type of anaemia does lead poisoning cause?
Microcytic anaemia
27
What mode of inheritance is sickle cell disease?
Autosomal recessive
28
In SCD, where does the mutation occur?
Beta globin gene
29
What does the presence of sickle cells cause disease?
They have a reduced deformability and are easily destroyed This causes occlusion of microcirculation and a chronic haemolytic anaemia of about 90g/dL
30
What are the different types of sickle cell disease?
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
31
What are the typical Hb proportions in sickle cell trait?
60% HbA and 40% HbS
32
What does sickle cell trait protect against?
Malaria
33
Where is SCT most prevalent?
Sub-Saharan Africa
34
What are the clinical features of SCT?
Generally asymptomatic - often have no abnormal clinical findings
35
Give three potential complications of SCT
``` Haematuria Decreased ability to concentrate urine Renal papillary necrosis Renal medullary cancer Splenic infarction Exertional rhabdomyolysis Sudden death: induced by hypoxia, dehydration or exercise ```
36
Which investigation is usually abnormal in SC trait?
Electrophoresis shows Hb AS
37
How should you advise a parent of a child with SCT?
Keep them adequately hydrated Avoid fluid loss and severe heat to prevent complications
38
When do symptoms usually begin in children with SCD?
3 - 6 months of age - HbF levels begin to fall
39
Why are kids with SCD more susceptible to infection?
Acute splenic sequestration
40
How does a child with SCD clinically present?
``` Anaemic Jaundice Pallor Lethargy Growth restirction/FTT General weakness Delayed puberty Splenomegaly ```
41
How does SCD usually cause anaemia?
1. Acute splenic sequestration 2. Transient red cell aplasia 3. Hyperhaemolysis in patients with severe infection
42
What are the different types of sickle cell crises?
Vaso-occlusive crisis Aplastic crisis Sequestration crisis Acute chest syndrome
43
What is an aplastic crisis?
Cessation of erythropoiesis cause severe anaemia
44
What often precipitates an aplastic crisis?
Parvovirus B19 infection
45
How might kids in aplastic crisis present?
Congestive cardiac failure
46
What might kids in aplastic crisis need?
Transfusion
47
What happens in a vaso-occlusive crisis?
There is obstruction of the microcirculation by sickle cells, causing ischaemia
48
What usually precipitates a vaso-occlusive crisis?
Cold Infection Dehydration
49
How might a child with a vaso-occlusive crisis present?
Pain - loin pain/abdominal pain/swollen painful joints Tachypnoea Fits and focal neurological signs Retinal occlusion
50
What can happen if the vaso-occlusive crisis involves large vessels?
Thrombotic stroke Acute sickle chest syndrome Placental infarction
51
Why might a child in vaso-occlusive crisis get abdominal pain?
Mesenteric sickling Bowel ischaemia
52
Why might a child in vaso-occlusive crisis get loin pain? Which other symptoms might accompany this?
Renal papillary necrosis Haematuria
53
What is a sequestration crisis?
Sequestration crises results from trapping of large amounts of red cells in the spleen and sometimes in the liver
54
Who does a sequestration crisis more commonly occur in?
Babies and infants
55
What is acute chest syndrome? What is its clinical presentation?
Vaso-occlusive crisis affecting the lungs Characterised by pleuritic chest pain, fever and new pulmonary infiltrate on CXR
56
What is the most common crisis in kids with SCD?
Vaso-occlusive crisis 2nd most common reason for admission is acute chest syndrome
57
How is a painful crisis usually managed?
Analgesia Fluids Warmth Rest Avoid cold exposure Look for cause e.g. infection and treat
58
Which drug can be used to reduce the frequency of crisis occurrence|?
Hydroxycarbamide
59
When are children with SCD given prophylactic penicillin?
By 3 months of age
60
Which infection are children with SCD more likely to get and specifically vaccinated against?
Pneumococcal
61
What is the management of acute chest syndrome?
CPAP Exchange transfusion Antibiotics - macrolide + IV cephalosporin
62
What investigation is done to monitor stroke in children with SCD?
Transcranial doppler USS
63
What other potential complication in kids with SCD requires immediate management?
Priapism (sustained erection) - hydration, analgesia, adrenaline and etilefrine
64
Give three other complications of SCD
``` Priapism Chronic pain Nocturnal enuresis Infection Stroke Cor pulmonale Gallstones Retinopathy Chronic leg ulcers Avascular necrosis of femoral/humeral head CKD Learning difficulties ```
65
How is diagnosis of sickle cell crisis made?
Positive sickling test wit HbS on electrophoresis
66
When would you urgently refer a child with SCD to hospital?
``` 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
What mode of inheritance are thalassaemias?
Autosomal recessive
68
What is the difference between thalassaemia minor and major?
Minor - heterozygous state - thalassaemia trait Those with the trait are suually asymptomatic or have mild microcytic anaemia Major - homozygous state - disease
69
When does thalassaemia present?
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
How does thalassaemia usually appear on FBC?
``` Microcytic hypochromic anaemia Low Hb Low MCV Low MCH Raised iron and ferritin ```
71
Which other clinical signs can be seen in thalassaemia?
``` 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
What are the main complications of alpha thalassaemia?
Hydrops fetalis | Perinatal death
73
What is done to prevent iron overload in thalassaemia patients?
Desferrioxamine bu subcutaneous infusion
74
What is doen to support RBC production in thalassaemia patients?
Folic acid and Vit C supplements | Regular blood transfusions
75
Give three complications of thalassaemia
``` Iron overload (therefore endocrine dysfunction) Transfusion risks Osteoporosis Gall stones/gout Hepatocellular carcinoma ```
76
What is the prognosis for severe beta thalassaemia?
80% die within five years
77
What is HSP?
IgA mediated autoimmune hypersensitivity vasculitis
78
Who is HSP more common in?
Female Caucasians 4-6 years old
79
Which four organs does HSP involve?
Skin Joints Gut Kidneys
80
What are the risk factors for HSP?
Infection: Group A strep, mycoplasma, EBV Vaccinations Environmental factors: allergens, pesticides, cold, insect bites
81
How does HSP present?
``` 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
How does the HSP rash develop?
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
How is HSP diagnosed? What do investigations show?
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
What are the renal complications of HSP?
Renal involvement is serious in 10% ESKD (<1%) Nephrotic syndrome Oliguria Hypertension
85
How should HSP be managed?
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
How should HSP patients with renal involvement be followed up?
If proteinuria, monthly No proteinuria - regular blood pressure checking and urinalysis
87
What are the other complications of HSP?
``` MI Pulmonary haemorrhage Pleural effusion Intussusception GI bleed Bowel infarction Seizures Mononeuropathies ```
88
What is the prognosis for HSP?
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
What is deficient in Haemophilia A and B?
A - Factor VIII | B - Factor IX
90
Which haemophilia is: 1. more common? 2. more severe?
A
91
What is the mode of inheritance of haemophilia?
X-linked recessive

Paediatrics and Child Health (11 decks)

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