Diagnosis

A tissue diagnosis is required before initiating definitive treatment, although one may have been performed before referral. Fresh and fixed tissue samples should be collected from the tissue biopsy for anatomical pathology and flow cytometry. Gene mutation testing may be of prognostic relevance in some cases.

Evaluate relevant organ function based on history, clinical examination and, where appropriate, laboratory or imaging investigations (cardiac, respiratory, renal, hepatic).

Staging and prognosis

Disease stage should be confirmed with an FDG-PET/CT scan and may include bone marrow evaluation. Other tests may be performed to evaluate the prognosis.

Genetic testing

Currently there are no genetic tests applicable to predict family risk of LGL.

Treatment planning

The multidisciplinary team should discuss all newly diagnosed patients with LGL prior to commencing any disease-directed therapy.

Research and clinical trials

See the OCP resources appendix and relevant steps for clinical trial resources relevant to LGL.

Communication

The lead clinician’s1 responsibilities include:

• discussing a timeframe for diagnosis and treatment options with the patient and/or carer
• explaining the role of the multidisciplinary team where indicated in treatment planning and ongoing care
• encouraging discussion about the diagnosis, prognosis, advance care planning and palliative care while clarifying the patient’s wishes, needs, beliefs and expectations, and their ability to comprehend the communication
• providing appropriate information and referral to support services as required
• communicating with the patient’s GP about the diagnosis, treatment plan and recommendations from multidisciplinary meetings
• explaining the principles of ‘watchful waiting’ if active surveillance is planned rather than commencing treatment.

Checklist

• Diagnosis has been confirmed
• Full histology obtained
• Performance status and comorbidities recorded
• Patient discussed at multidisciplinary meeting and decisions provided to the patient and/or carer
• Clinical trials considered
• Supportive care needs assessed and referrals to allied health services actioned as required
• Referral to support services (such as Cancer Council, Leukaemia Foundation, Lymphoma Australia)
• Treatment costs discussed with the patient and/ or carer

Timeframe

Referrals should be triaged on the basis of presence of indicators of concern and the timing of diagnostic investigations should be guided by the initial severity of symptoms. Staging should be completed within 4 weeks.

All cases should be reviewed at a multidisciplinary meeting.

1 Lead clinician – the clinician who is responsible for managing patient care. The lead clinician may change over time depending on the stage of the care pathway and where care is being provided.