Lochie Teague

Service Clinical Director

Starship Children’s
Hospital
How do Pacific people with childhood cancer
compare with others in NZ and around the world?
Incidence by Prioritised Ethnicity
Maori Pacific Peoples Non-Maori/non
Pacific
No. Age No. Age No. Age
Case Standardised Case Standardised Case Standardised
s rate (95% CI) s rate (95% CI) s rate (95% CI)
All 262 131.1 131 173.4 936 158.1
(115.2 – 146.9) (143.7 – 203.1) (147.9 – 168.2)
Incidence of Common Cancers by Ethnicity
Age Adjusted Incidence (per 1,000,000 per year)
Non-
Maori Pacific Peoples Maori/Non-
Pacific
Leukaemia 46.5 71.5 52.4
CNS tumours 26.0 29.1 35.5
Lymphoma 11.1 11.9 14.0
Neuroblastoma 10.0 5.3 11.0
Soft Tissue
8.5 3.2 12.5
Sarcomas
Germ Cell
8.5 15.9 5.2
Tumours
Cumulative Survival by Age
100
90
80
70
60
50 0-4 years
5-9 years
40
10-14 years
30
20
10
0
0 1 2 3 4 5 6 7 8 9 10
Time since diagnosis (years)
Survival by Prioritised Ethnicity

100
90
80
Relative survival (%)

70
60
50
40
30
20
10
0
0 1 2 3 4 5 6 7 8 9 10
Time since diagnosis (years)
Maori Pacific Peoples Non-Maori/Pacific Peoples
5 year Relative Survival by Age and Ethnicity
Total 0-14
0-4 years 5-9 years 10-14 years
years
Five-year Five-year Five-year Five-year
relative survival relative survival relative survival relative survival
(95% CI) (95% CI) (95% CI) (95% CI)

81.3 72.8 74.2 76.9

Maori
(72.3 - 87.6) (59.8 - 82.2) (62.5 - 82.8) (71.0 - 81.8)
Pacific 73.8 90.2 79.9 81.4
Peoples (58.4 - 84.3) (75.7 - 96.3) (61.9 - 90.1) (73.1 - 87.3)
Non-Maori/ 84.2 80.1 79.3 81.7
Pacific Peoples (80.2 - 87.5) (74.0 - 85.0) (73.7 - 83.9) (79.0 - 84.2)
82.8 79.9 78.4 80.7
Total
(79.4 - 85.8) (74.9 - 83.9) (73.7 - 82.3) (78.4 - 82.9)
 May 2016 PI ALL-1 Protocol
 Sept 2016 PI ALL-2 Protocol

Major initial objective met

 Proven that therapy can safely be given in Tonga, Fiji

and potentially Samoa
Suspected Diagnosis

 ALL
- 25-30% of all childhood cancers
- Symptoms: fever, lassitude (“unwell”), pallor, bone
pain
- Signs: Anaemic, Neutropenia, thrombocytopenia,
lymphoadenopathy, splenomegaly,
hepatosplenomegaly
 FBC
- 80% + have WBC > 50
- 80% + anaemic
- 80% + thrombocytopenia
Initial Treatment

 Blood transfusion
 Platelet transfusion
 Antibiotics
 Fluids
 Allopurinol
Confirm Diagnosis
B lineage ALL

 Blood and/or Bone Marrow

- Once diagnosis is made need to urgently anticipate
referral and arrival in NZ by Day 8. If coming from
Tonga/Samoa for Fiji, initial induction Day 8. As per
protocol

- At this point have already selected the majority of

children who have a 75% chance of cure with
completed therapy as per PI ALL-2
Current trends in USA and around the world

 Sophisticated stratification
 Minimal residual disease (MRD) monitoring response to
initial phases of therapy
 Trialling of new agents
- Tyrosine Kinase Inhibitors
- Blinatumomab
- CAR T cells
 Bone marrow transplant rare in first remission – but
commonly used in second remission provided child
responds to 2nd line chemotherapy
Key points of note in PI ALL-2 protocol

Similar initial phases, but delayed intensification

(Reinduction/reconsolidation) phase added before
maintenance therapy.

Expect
- toxicity – antibiotics, anti fungal, blood product
support, anti emetic drugs
- infection, mainly bacterial, occasionally fungal
Intrathecal therapy is only with intrathecal
methotrexate *safety policy*

Need to recognise that any fever,unwellness in a child on

chemotherapy maybe due to bacterial infection requires
urgent assessment and starting antibiotics essential

Febrile neutropenia protocol

Hygiene precaution
Asparaginase

 Need to recognise adverse reaction and Anaphylaxis

reactions
 Need to treat Anaphylaxis as emergency

Adrenalin 1:10,000
0.1ml/kg i.m
O2
Antihistamines, Hydrocortizone
Remember

 (Age + 4yrs) x2 = weight in kgs

- But should have drug doses worked out ahead of time
- Needles, drugs and equipment nearby child for
resusitation.
- Observe for 1 hour with asparginase injection
If Asparaginase Anaphylaxis

 Don’t give anymore of same product ever again

 If occurs after several doses don’t bother with switch to

Erwinase due to cost and any relative additional
benefit hard to quantify.
Pneumocystis Jirovecii (Carinii)
Prophylaxis and Pneumonia (PCP)

 Standard treatment: cotrimoxizole twice daily, 2 days /

wk
- But twice daily 1 day/wk may be sufficient

 Consider switching to pentamidine 4mg/kg by iv

infusion

Pentamidine costs $36NZD per 300mg

Maintenance Therapy
6 mercaptopurine (6mp)

 Essential to success of therapy

 Given on empty stomach (at last 2 hours after last
meal)
 Same time of day every day
 If child vomits within 1 hour, regardless of whether
tablet is seen, repeat dose
Methotrexate

 Beware photosensitivity rashes

 Ulcers
 Can affect renal and liver function
End of therapy

 Bone Marrow?
 Frequency of follow ups
Communication –vital!
 At Starship our whole team meets 3x a week to discuss
all inpatients.
 We discuss difficulties with outpatients as required.
 And any time during routine hours to discuss
maintenance issues with shared care doctors/nurses
Acute Myeloid Leukaemia

 Approximately 20% of all cases of leukaemia in

children
- Can not be distinguished from ALL on clinical grounds
- FBC or bone marrow
 Myeloperoxidase positive
 Auer rods if present indicate AML (or APML)

Poor prognosis unless very intensive chemotherapy

Acute Promyelocytic Leukaemia
(APML or AML-M3)

 10 – 15% of AML
 Disseminated intravascular coagulation (DIC)
 Expect a cure rate of 70%
 Diagnosis made on FBE/Bone Marrow
- Abnormal Promyelocytes
 Start ATRA immediately diagnosis suspected.
Dexamethazone important for retinoic acid
syndrome (RAS)
(Differentiation syndrome (DS))

 Or a rising WBC > 5

- And continue until WBC <5
- Withhold ATRA if RAS (DS) develops
APML

 Do not perform lumber puncture (Not needed)

- Only exception being if patient had CNS bleed then only
after DIC settled to perform LP + IT Therapy
- Dex 2.5mg/M2/dose twice daily o/iv
 Hydroxyurea 15mg/kg/dose orally 4x day (if WBC rises
to >50 double dose)
 Pseudotumour cerebri, headaches
- Need CT Head
- High CSF pressure
Chronic Myeloid Leukaemia
(CML)
• Rare
• FBC high neutrophil and myelocyte count
• Splenomegaly
• Hydroxyurea
• Imatinib (Gleevec)