Guidelines on the Diagnosis and Management of Multiple Myeloma
2. Myeloma - the context
Epidemiology and Incidence
Myeloma is a plasma cell tumour with an annual United Kingdom incidence of 40 per million (about 2500 new cases) per annum; at any one time there will be 10 - 15,000 patients with the condition. The median age at diagnosis is 60 - 65 years; fewer than 2% of myeloma patients are under 40 years old at diagnosis. It has a higher incidence in Afro-Caribbean ethnic groups compared to Caucasians; little else is known specifically about its epidemiology.
Most cases present de novo; a minority evolve from Monoclonal Gammopathy of Undetermined Significance (MGUS).
Clinical Presentation
Clinical presentation is varied. Common presentations of myeloma include bone pain, recurrent or persistent infection, anaemia, renal impairment, or a combination of these. Some patients are asymptomatic, abnormalities being identified on blood tests being carried out for other clinical reasons.
Presenting features, which require urgent specialist referral, include:
- Bone destruction symptoms:
- persistent, unexplained backache associated with loss of height and osteoporosis (especially in men and pre-menopausal women)
- symptoms suggestive of spinal cord/nerve root compression
- Compromised immunity and/or bone marrow function:
- recurrent or persistent bacterial infection
- anaemia, typically normochromic
- leucopenia and/or thrombocytopenia
- Persistent elevation of ESR or plasma viscosity
- Evidence of impaired renal function
- Hypercalcaemia
Investigations available in primary care which can identify suspected cases of myeloma include:
- ESR or plasma viscosity
- full blood count
- serum urea & electrolytes
- serum calcium
- serum protein electrophoresis
- serum immunoglobulins
- urine sample to test for free light chains (Bence - Jones protein)
- standard X-ray imaging of bones, particularly the axial skeleton
NB. Radionucleide bone scanning is not usually helpful
in the diagnosis of myeloma.
Appropriate Setting for the Management of Myeloma
A consultant haematologist or oncologist, who is part of an approved Cancer network in accordance with UK NHS strategy, should lead the care of patients with multiple myeloma. Effective and high-quality care in myeloma requires a multi-specialty and multi-disciplinary team familiar with the range of clinical problems likely to be encountered.
There are currently a number of government initiatives focused on improving standards of care including the NHS Cancer Plan. Clinicians managing patients must be aware of these initiatives and develop strategies to achieve these standards locally.
The centre should be capable of achieving the 2-week government standards for referral. It needs to maintain a database of cases for registration and clinical audit. A minimum data set for myeloma registration and audit is included as Appendix 1. Improved registration and systems to facilitate this are part of the NHS Cancer strategy.
The following represent the core range of essential accessible expertise and services, which may be available locally or in a neighbouring hospital. There should be clear policies and protocols for access to these services.
- Haematology/Oncology nurse specialists
- Clinical pathology
- Diagnostic radiology
- Pharmacy facilities and expertise for dispensing cytotoxic drugs
- Renal services, including rapid access to haemodialysis
- Clinical oncology
- Neurosurgery
- Orthopaedic surgery
- Accredited bone marrow / stem cell transplant centre
- Primary care liaison
- Palliative care physicians / nurses
- Physiotherapy/rehabilitation
- Administrative support for case registration, audit & clinical trials
- Social services and financial advice
- Patient support group
- Written information as well as guidance on access to electronic information
- Treating centres must meet standards required for Level 1 care (British Committee for Standards in Haematology, 1995)
- Where therapy involves autologous stem cell transplantation this must be done in an EBMT accredited centre, equipped to provide Level 3 care for haematological malignancies, as defined by the British Committee for Standards in Haematology (1995)