Paediatric Haem-Onc Flashcards

1
Q

Name the most common cancer in children.

A

Acute lymphoblastic leukaemia

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2
Q

Describe the pathophysiology of ALL.

A

Malignant proliferation of immature blast cells, usually B cell precursors, which infiltrate bone marrow and other organs of reticuloendothelial system.

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3
Q

Describe the symptoms/signs in ALL and explain why these occur.

A

Usually presents insidiously over several weeks but may present very rapidly.

  1. General
    - malaise
    - anorexia
  2. BM infiltration
    - anaemia: fatigue, pallor
    - neutropenia: infections
    - thrombocytopaenia: bruising, petechiae, nose bleeds
    - bone pain (25%)
  3. reticulo-endothelial infiltration
    - hepatosplenomegaly
    - lymphadenopathy
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4
Q

Name 2 risk factors for the development of ALL.

A
  1. age: 2-6 years

2. Down syndrome

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5
Q

Which investigations would you perform on a child with suspected ALL to confirm Dx?

A
  1. Bloods
    - FBC: anaemia, thrombocytopaenia, neutropenia
    - peripheral blood smear: blast cells +/- RBC abnormalities
    - clotting screen: 10% have DIC at time of diagnosis
  2. BMAT (diagnostic)
    - >25% malignant blast cells
    - for morphology, immunophenotyping + cytogenetics
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6
Q

Which Ix would you perform on a child with suspected/confirmed ALL to assess for disease spread?

A
  1. CXR: to identify mediastinal mass characteristic of T cell disease
  2. abdo. USS: may show renal enlargement from leukaemic infiltration or uric acid nephropathy as well as intra-abdo. adenopathy
  3. LP: to ID CSF disease
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7
Q

Which supportive treatments are required in a cild being managed for ALL?

A
  1. correction of any cytopaenias e.g. RBC or platelet transfusions
  2. additional hydration + allopurinol (to protect renal function against effects of rapid cell lysis)
  3. co-trimoxazole on 2-3 consecutive days each week (prophylaxis against Pneumocystis jirovecii pneumonia)
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8
Q

What is the definitive treatment for ALL?

A

Combination chemotherapy:
1. induction (4 wks): steroids + chemo (+ intrathecal chemo if CNS +ve)

  1. consolidation: continued systemic chemo + intrathecal chemo (+ CNS radiotherapy if CNS +ve)
  2. maintenance (2-3 yrs): continued systemic chemo +/- intrathecal chemo.
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9
Q

what is the prognosis for ALL in children? what are high risk factors?

A

80% survival with treatment.

Poor prognostic features:

  • age <1 yr or >10 yrs
  • WCC >50
  • some tumour cytogenetic/molecular abnormalities
  • slow response to initial chemo
  • detectable minimal residual disease after induction therapy
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10
Q

a 5 year old girl presents with a 7 day history of frequent bruising and nose bleeds. O/E she has a purpuric rash. She had a viral URTI 2 weeks ago. What is the diagnosis and what is the pathophysiology of this? How would you manage her?

A

IMMUNE THROMBOCYTOPAENIC PURPURA

  • most commonly occurs following a viral infection
  • production of autoimmune IgG against platelet cell membrane antigen causing platelet destruction

Management:

  • advice (e.g. avoid contact sport) and observational monitoring
  • avoid aspirin and NSAIDs
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11
Q

How would you treat a child presenting with severe ITP (i.e. significant bleeding)?

A

1st line: prednisolone, IV immunoglobulin

2nd line: rituximab, high dose dexamethasone

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12
Q

what is the prognosis for childhood ITP?

A

Up to 80% completely recover within 6 months.
Some will develop chronic thrombocytopaenia, but most will require no Tx.
<0.5% develop intracranial haemorrhage.

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13
Q

what is your differential diagnosis for a child who presents with a petechial/purpuric rash?

A

Thrombocytopaenic causes:

  1. ITP
  2. leukaemia
  3. DIC

Non-thrombocytopaenic causes:

  1. Henoch-Schonlein purpura
  2. sepsis (meningococcal/viral)
  3. trauma
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