1.3.1 Screening recommendations

There are no screening tools for early detection of newly diagnosed CAYA acute leukaemia at the moment. In CAYA with an identified cancer predisposition, a specialist may recommend screening full blood count examinations or bone marrow examination. Routine full blood count examination is not indicated for asymptomatic siblings of those diagnosed with CAYA acute leukaemia.

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STEP 2: Presentation, initial investigations and referral

Because CAYA acute leukaemia is rare it represents a major diagnostic challenge for primary healthcare professionals including GPs and emergency physicians. It is important to recognise parental concern and the need to escalate investigations, particularly after repeated visits to healthcare professionals. The following signs and symptoms should be investigated and may warrant the consideration of…

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STEP 1: Prevention and early detection

Prevention Although risk factors have been identified, the cause of CAYA acute leukemia is unknown. There is currently no evidence that lifestyle plays a role. It is important to ensure the patient and their family and/or carer are aware of this to avoid feeling responsible for the illness. Risk factors The risk factors for developing…

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STEP 3: Diagnosis, staging and treatment planning

Diagnosis and staging Perform pre-treatment medical investigations on the day of presentation to the specialist cancer service. The diagnostic laboratory investigations should be performed as follows. Urgent pathway For urgent cases, if it is safe to do so, a diagnostic bone marrow aspirate and lumbar puncture should be performed on the day of presentation. Urgent…

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STEP 4: Treatment

Intent of treatment for CAYA acute leukaemia is curative. Treatment options Chemotherapy with or without immunotherapy is the key component for treating CAYA acute leukaemia. Radiation therapy has a role in some CAYA acute leukaemia patients with testicular disease or overt central nervous system disease. Radiation therapy is used as part of the conditioning regimen…

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STEP 5: Care after initial treatment and recovery

Provide a treatment and follow-up summary to the patient, family and/or carer and GP outlining: the diagnosis, including tests performed and results treatment received (types and date) current toxicities (severity, management and expected outcomes) interventions and treatment plans from other health professionals potential long-term and late effects of treatment and care of these supportive care…

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STEP 7: End-of-life care

Consider a referral to palliative care. Ensure an advance care directive is in place. Communication The lead clinician’s responsibilities include: being open about the prognosis and discussing palliative care options with the patient establishing transition plans to ensure the patient’s needs and goals are considered in the appropriate environment. Checklist Supportive care needs assessed and…

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STEP 6: Managing relapsed or refractory disease

Detection Most residual or recurrent disease will be detected via routine follow-up or by the patient presenting with symptoms. Treatment Evaluate each patient for whether referral to the original multidisciplinary team is appropriate. Treatment will depend on the extent of disease, previous management and the patient’s preferences. Advance care planning Advance care planning is important…

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Resources

Visit the guides to best cancer care webpage <www.cancercareguides.org.au> for consumer guides. Visit the OCP webpage <www.cancer.org.au/OCP> for the optimal care pathway and instructions on how to import these guides into your GP software. Endorsed by: ALLG <www.allg.org.au> ANZCHOG <www.anzchog.org> ANZTCT <www.anztct.org.au> Cancer Council <www.cancer.org.au> Canteen <www.canteen.org.au> HSANZ <www.hsanz.org.au> Leukaemia Foundation <www.leukaemia.org.au> Redkite <www.redkite.org.au>

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