STEP 2: Presentation, initial investigations and referral
This step outlines the process for the general practitioner to initiate the right investigations and refer to the appropriate specialist in a timely manner. The types of investigations the general practitioner undertakes will depend on many factors, including access to diagnostic tests, the availability of medical specialists and patient preferences.
MM can present with many otherwise unexplained, non-specific symptoms and/or blood test abnormalities, as well as end-organ complications or related organ tissue injury.
End-organ complications that are often associated with MM include (Quach & Prince 2019):
- enhanced bone loss
- renal failure
- hypercalcaemia
- immune suppression
Signs and symptoms include (Kyle et al. 2003):
- fatigue and generalised weakness
- bone pain or pathological fracture (broken bone often with minimal trauma)
- frequent infections
- unintentional weight loss
- symptoms of hypercalcaemia including mental fogginess and/or confusion, new-onset constipation and/or abdominal pain and increased thirst
- new-onset back pain, particularly with neurological symptoms such as leg weakness, loss of bladder or bowel control, or loss of sensation
- symptoms of hyperviscosity (easy bruising, bleeding gums, cloudy vision), although this is rare. Blood test abnormalities that may suggest MM include:
- anaemia
- elevated creatinine
- hypercalcaemia
- elevated ESR in the absence of a known infection or inflammation
- an increased protein–albumin gap in the absence of infection or inflammation
- presence of a paraprotein on serum protein electrophoresis
- elevated serum kappa or lambda free light chains with abnormal kappa–lambda light chain
The presence of multiple signs and symptoms, particularly in combination with other underlying risk factors, indicates an increased risk of MM.
Presenting symptoms should be promptly assessed. If there are no severe symptoms or severe blood test abnormalities, work-up can be initiated in general practice.
If there is high suspicion of MM after clinical assessment, the general practitioner should promptly
refer the patient to a haematologist or specialist centre to confirm the diagnosis.
In cases where clinical suspicion is lower, general practitioner examinations and investigations should include the following.
Blood test to identify paraproteins and/or elevated light chains that may indicate underlying MM:
- serum protein electrophoresis (SPEP) and immunofixation (IF)
- serum kappa and lambda free light chain (SFLC), and light chain ratio. Blood and urine tests that may indicate end-organ dysfunction:
- full blood count, differential
- creatinine, urea and electrolytes
- liver function tests, calcium, magnesium, phosphate, urate
- urine dipstick full ward test to look for
X-ray or computed tomography (CT) imaging of painful areas to assess for fractures, lytic lesions and/or soft tissue plasmacytomas may also be warranted.
When moderate or severe symptoms are present in MM as a differential diagnosis, the general practitioner should review promptly (within two days) to avoid missing a medical emergency such as severe hypercalcaemia, renal failure or spinal cord compression.
In non-urgent cases where investigations are done in general practice, the general practitioner should have results, review the patient and finalise a path of action within four weeks.
If the diagnosis of MM is confirmed or the results are inconsistent or indeterminate, the general practitioner must refer the patient to an appropriate specialist (haematologist) or a health service with a multiple myeloma-specific multidisciplinary team to make the diagnosis.
Patients should be enabled to make informed decisions about their choice of specialist and health service. General practitioners should make referrals in consultation with the patient after considering the clinical care needed, cost implications (see referral choices and informed financial consent on Page 9), waiting periods, location and facilities, including discussing the patient’s preference for health care through the public or the private system.
Referral for suspected or diagnosed MM should include the following essential information to accurately triage and categorise the level of clinical urgency:
- important psychosocial history and relevant medical history
- family history, current symptoms, medications and allergies
- results of current clinical investigations (imaging and pathology reports)
- results of all prior relevant investigations
- notification if an interpreter service is
Many services will reject incomplete referrals, so it is important that referrals comply with all relevant health service criteria.
If access is via online referral, a lack of a hard copy should not delay referral.
The specialist should provide timely communication to the general practitioner about the consultation and should notify the general practitioner if the patient does not attend appointments.
Aboriginal and Torres Strait Islander patients will need a culturally appropriate referral. To view the optimal care pathway for Aboriginal and Torres Strait Islander people and the corresponding quick reference guide, visit the Cancer Australia website . Download the consumer resources – Checking for cancer and Cancer from the Cancer Australia website.
The timing of specialist referral is guided by clinical severity and the presence of end-organ damage.
Indicators of end-organ damage in patients with MM include (Quach & Prince 2019; Rajkumar et al. 2014):
- hypercalcaemia: corrected serum calcium 0.25 mmol/L above the upper limit of normal or higher than 2.75 mmol/L
- renal impairment: creatinine clearance 177 μmol/L (> 2 mg/dL)
- anaemia: haemoglobin below 100 g/L or 20 g/L below the lower limit of normal
- bone lesions: one or more osteolytic lesions on skeletal radiography, CT or positron emission tomography (PET) CT.
Patients with evidence of end-organ damage should be seen by a haematologist as soon as possible, ideally not longer than one week.
Patients without overt end-organ damage should ideally be seen by a haematologist within four weeks.
Patients with severe hypercalcaemia, renal failure, symptoms of hyperviscosity, severe new-onset back pain or acute neurological symptoms should be immediately referred to a haematologist or emergency department.
The patient’s general practitioner should consider an individualised supportive care assessment where appropriate to identify the needs of an individual, their carer and family. Refer to appropriate support services as required. See validated screening tools mentioned in Principle 4 ‘Supportive care’.
A number of specific needs may arise for patients at this time:
- assistance for dealing with the emotional distress and/or anger of dealing with a potential cancer diagnosis, anxiety/depression, interpersonal problems and adjustment difficulties
- management of physical symptoms, particularly infections and pain
- encouragement and support to increase levels of exercise (Cormie et al. 2018; Hayes et al. 2019) provided that it’s safe to do so.
For more information refer to the National Institute for Health and Care Excellence 2015 guidelines, Suspected cancer: recognition and referral .
For additional information on supportive care and needs that may arise for different population groups, see Appendices A, B and C.
The general practitioner is responsible for:
- providing patients with information that clearly describes to whom they are being referred, the reason for referral and the expected timeframes for appointments
- requesting that patients notify them if the specialist has not been in contact within the expected timeframe
- considering referral options for patients living rurally or remotely
- supporting the patient while waiting for the specialist appointment (Cancer Council and Myeloma Australia nurses are available to act as a point of information and reassurance during the anxious period of awaiting further diagnostic information).
More information
A range of disease-specific support organisations may be available for patients. These include:
- Cancer Council: 13 11 20
- Leukaemia Foundation: 1800 620 420
- Myeloma Australia: 1800 693
Refer to Principle 6 ‘Communication’ for communication skills training programs and resources.