Establish intent of treatment

• Curative
• Anti-cancer therapy to improve quality of life and/or longevity without expectation of cure
• Symptom palliation.

Watchful waiting (WW) is appropriate for asymptomatic FL and MZL in stage II (which is not suited to radiotherapy) and advanced-stage FL and MZL (stages III and IV). The frequency of clinical review is based on the tempo of the disease, the comfort of the patient with a WW approach and the likelihood that they will recognise and re-present at signs of relapse. WW can cause significant psychological distress, so referral to a psycho-oncology service experienced in lymphoma is recommended.

Indolent MCL, commonly a leukaemic presentation, indicated by lack of symptoms and low tumour burden, can be followed with WW.

Infection eradication of Helicobacter pylori may induce remission for gastric MALT without the need for radiotherapy.

Radiation therapy has an important role in specific scenarios within indolent lymphoma subtypes:

• potentially curable for early-stage FL (stage I and stage II in which lymph nodes are contiguous)
• potentially curable for early-stage MZL (including gastric, cutaneous, unilateral and bilateral orbital MALT, and other localised MZLs)
• used for symptom control in advanced- stage LGL, where lymphoma is impairing quality of life.

Systemic therapy is appropriate in most patients with FL and MZL with non-contiguous stage II or stage III–IV disease who are symptomatic. Systemic therapy is most commonly used with chemotherapy and anti-CD20 monoclonal antibody. MCL causing symptoms should be treated with systemic chemotherapy and anti-CD20 monoclonal antibody combination, incorporating high-dose cytarabine in younger, fitter patients, followed by consolidation with autologous stem cell transplantation.

Surgery is a potential first-line treatment option (splenectomy) for splenic MZL.

Palliative care

Early referral to palliative care can improve quality of life and in some cases survival. Referral should be based on need, not prognosis.

For more information, visit the Palliative Care Australia website <www.palliativecare.org.au>.

Communication

The lead clinician and team’s responsibilities include:

• discussing treatment options with the patient and/or carer including the intent of treatment as well as risks and benefits
• discussing advance care planning with the patient and/or carer where appropriate
• communicating the treatment plan to the patient’s GP
• helping patients to find appropriate support for exercise programs where appropriate to improve treatment outcomes.

Checklist

• Intent, risk and benefits of treatment discussed with the patient and/or carer
• Treatment plan discussed with the patient and/or carer and provided to GP
• Supportive care needs assessed and referrals to allied health services actioned as required
• Early referral to palliative care considered and advance care planning discussed with the patient and/or carer

Timeframe

Treatment may start after a long WW period; however, where there are symptoms of concern treatment should start urgently.

In FL and MZL, the decision of when to start systemic therapy is guided by the internationally accepted standard criteria and treatment should begin within 4 weeks.

Most symptomatic MCL patients should begin treatment with systemic therapy within 2 weeks of completing staging.