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Paediatrics > Haematology/Oncology > Flashcards

Haematology/Oncology Flashcards

1
Q

What are the different types of tumours which are commonly seen in children?

A
  • Acute leukaemia
  • Brain/spinal cord
  • Neuroblastoma
  • Lymphoma
  • Wilms tumour
  • Bone tumour
  • Retinoblastoma
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2
Q

How common is cancer in children?

A

1 in 600

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3
Q

What disease processes can pre-dispose to the development of cancer?

A
  • Down
  • Fanconi
  • Beckwith Weiderman Syndrome
  • Li-Fraumeni Familial Cancer Syndrome - p53
  • Neurofibomatosis
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4
Q

What environmental factors can cause cancer?

A
  • Radiation
    • Ionising
    • Non-ionising - Sun
  • Viral infection
    • EBV
    • Hepatitis B
    • HIV
  • Iatrogenic
    • Radiotherapy
    • Chemotherapy
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5
Q

What is the most common form of leukaemia in children?

A

Acute lymphoblastic leukaemia - 80%

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6
Q

What is the clinical presentation of ALL in children?

A
  • Malaise/anorexia
  • Pallor, lethargy - Anaemia
  • Infection - neutropenia
  • Bruising, petechiae, nose bleeds - thrombocytopenia
  • Hepatosplenomegaly
  • Lymphadenopathy - painless lumps
  • Fever
  • Headache, vomiting, nerve palsies - CNS involvement
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7
Q

How would you investigate if you suspected ALL?

A
  • FBC - anaemia, variable WCC, decreased platelets
  • Bone Marrow Biopsy - primarily blast cells
  • Blood film - normochromic, normocytic anaemia
  • CXR - mediastinal masses
  • CSF - pleocytosis, increased protein, decreased glucose
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8
Q

What are the common sites for brain tumours in children?

A
  • Cortex - astrocytoma
  • Cerebellum - medulloblastoma, astrocytoma, ependymoma
  • Brainstem - glioma
  • Spinal cord - Astrocytoma, ependymoma
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9
Q

What are common presentations in children with brain tumours?

A
  • Presistent/recurrent vomiting
  • Balance, coordination, walking
  • Behavioural change
  • Abnormal eye movements
  • Seizures
  • Abnormal head position
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10
Q

What are symptoms of brain tumours which are specifically seen in children/adolescents?

A
  • Persistent/recurrent headache
  • Blurred/double vision
  • Lethargy
  • Delayed/arrested puberty
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11
Q

What are symptoms of brain tumours in infants?

A
  • Developmental delay/regression
  • Progressive increase in head circumference
  • Bulging fontanelles
  • Separation of sutures
  • Lethargy
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12
Q

How would you investage a suspected brain tumour?

A

MRI scan

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13
Q

How does hodgkin’s lymphoma present in children?

A
  • Painless lymphadenopathy - frequently around the neck
  • B-symptoms - pruritis, fever, weight loss, sweating
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14
Q

What is a neuroblastoma?

A

A tumour that arises from neural crest tissue in the adrenal medulla and sympathetic nervous system. It can be benign (ganglioneuroma) to highly malignant (neuroblastoma).

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15
Q

What are common presenting symptoms of neuroblastoma?

A
  • Pallor
  • Weight loss
  • Abdominal mass
  • Hepatomegaly
  • Bone pain
  • Limp
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16
Q

Where is the most common site for neuroblastoma to occur?

A

Adrenal glands - leads to abdominal mass

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17
Q

What is a wilm’s tumour?

A

Nephroblastoma

A malignant tumor containing metanephric blastema, stromal and epithelial derivatives. Thought to be caused by mutation on the 11q13 chromosome

18
Q

How does Wilm’s Tumour tend to present?

A
  • Large abdominal/flank mass
  • Haematuria
  • Hypertension - renin secretion
  • Anorexia
  • Abdominal pain
19
Q

How would you diagnose Wilm’s Tumour?

A
  • US
  • MRI
20
Q

What is the most common bone tumour in young children?

A

Ewing’s Sarcoma

21
Q

What is retinoblastoma?

A

Malignant tumour of the retinal cells which can arise from mutations in the RB1 gene or the MYCN gene.

22
Q

How does retinoblastoma present?

A

White pupillary reflex

23
Q

What is tumour lysis syndrome?

A

Refers to the constellation of metabolic disturbances that may follow the initiation of cancer treatment

24
Q

How does tumour lysis syndrome occur?

A

Malignant cells have high turnover rate -> produce a greater amount of nucleic acid products (which transform to uric acid) and phosphate.

TLS is caused by the rapid destruction of tumour cells, usually in response to chemotherapy. Release of intracellular contents into bloodstream results in an elevation in serum levels:

  • Uric acid
  • Potassium
  • Phosphate -> reduction in the level of calcium
25
Q

In TLS, why does acute renal failure occur?

A

Kidney’s filtering ability becomes saturated

  • Hyperuricaemia + acidic urine + reduced urinary flow = uric acid crystals + renal tubular obstruction + decline in renal function.
  • Hyperphosphataemia = nephrocalcinosis + urinary obstruction
26
Q

What are symptoms of tumour lysis syndrome in children?

A

After initial treatment - typically 7 days

  • Nausea/Vomiting
  • Anorexia/Lethargy
  • Diarrhoea
  • Muscle weakness/paraesthesia/cramps
  • Laryngeal spasm.
27
Q

What are signs of Tumour Lysis Syndrome?

A
  • Related to characteristics/complications
  • Hyper/hypotension
  • Oliguria/anuria/haematuria
  • Cardiac arrhythmias - syncope, chest pain, or dyspnoea
  • Hypocalcaemia signs - tetany, Chvostek sign, Trousseau sign, or seizures
28
Q

How would you manage a child who is showing signs of tumour lysis syndrome?

A
  • ECG Monitoring
  • Hyperhydrate - 2.5l/m2
  • Diuresis
  • Treat hyperkalaemia
  • Watch urate and PO4
29
Q

What is important to remember when treating a child with Tumour lysis syndrome?

A

NEVER GIVE THEM K+

30
Q

How do you treat hyperkalaemia in the context of tumour lysis syndrome?

A
  • Calcium gluconate - cardioprotective agent - reducing heart muscle excitability and resolving many changes in heart rhythm seen on an ECG
  • Salbutamol - lowers blood levels of K+ by promoting its movement into cells
  • Insulin/dextrose - drives potassium back into the cell
31
Q

How would you manage increased urate levels in a child with Tumour lysis syndrome?

A

Decrease uric acid

  • Allopurinol - acts on xanthine oxidase
  • Rasburicase - acts as urate oxidase -> converts Uric acid to allantoin - more likely to use in context of large tumour
32
Q

What is the definition of febrile neutropenia?

A

_Neutrophils < 0.5 x 109/L and Fever* > 38.0°C_

In addition, patients for whom there is a clinical suspicion of sepsis in the absence of fever should be treated as potentially bacteraemic e.g. unexplained abdominal pain or generally unwell.

33
Q

How would you investigate suspected febrile neutropenia?

A
  • Bloods - FBC, CRP, Serology
  • Cultures - BCs, Swab, Stool, Urine
34
Q

How would you treat febrile neutropenia?

A
  • Piperacillin/tazobactam
  • Meropenem - if penicillin allergy
  • Add Gentamicin - if advised by consultant.
  • Add Teicoplanin - if fever and/or rigors after line flushed earlier in day or soon after new line inserted
35
Q

How would you manage a child with persistent febrile neutropenia after initial treatment?

A

Consider viral or fungal cause

  • Aciclovir?
  • Amphotericin?
36
Q

What are features of cord compression in a child with a tumour?

A
  • Ambulatory problems - Paraplegia
  • Spine tenderness
  • Spinchter distrubance
37
Q

How would you treat cord compression in children with cancer?

A
  • Dexamethasone
  • Chemotherapy
  • DXT - adjuvant radiotherapy
  • Surgery
38
Q

What is the abnormal red reflex seen in retinoblastoma called?

A

Leucocoria

39
Q

What gene is at fault in retinoblastoma, and on what chromosome is it on?

A

RB1 gene on chromosome 13

40
Q

How does retinoblastoma present?

A
  • Leucocoria
  • Squint
  • Cellulitis
  • Red Eye
  • Painful eye
  • Poor vision
41
Q

What secondary malignancies can Retinoblastoma develop?

A
  • Malignant melanoma
  • Sarcoma
  • Brain tumours
  • Leukaemia
  • Osteosarcoma

Paediatrics (27 decks)

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