Referral to emergency
If any of the following are present or suspected, please refer the patient to the emergency department (via ambulance if necessary) or seek emergent medical advice if in a remote region.
- polycythaemia with associated symptoms of ischaemia for example neurological symptoms, chest discomfort or vision changes
- any signs and symptoms of an acute myocardial event or acute neurological event
Please contact the on-call registrar to discuss your concerns prior to referral.
For clinical advice, please telephone the relevant specialty service.
Central Adelaide Local Health Network
- Royal Adelaide Hospital (08) 7074 0000
- The Queen Elizabeth Hospital (08) 8222 6000
Northern Adelaide Local Health Network
- Lyell McEwin Hospital (08) 8182 9000
- Modbury Hospital (08) 8161 2000
Southern Adelaide Local Health Network
- Flinders Medical Centre (08) 8204 5511
- Noarlunga Hospital (08) 8384 9222
Inclusions
- suspicion of primary polycythaemia
- JAK2 mutation positive – indicative of myeloproliferative neoplasm
- haematocrit persistently (3 months apart) above 0.52 in men and 0.48 in women and no secondary cause
Exclusions
- polycythaemia due to a secondary cause, for example chronic hypoxia, renal disease, smoking, androgen use
Triage categories
Category 1 (appointment clinically indicated within 30 days)
- haemoglobin (Hb) > 200g/L and asymptomatic
- haematocrit (HCT) > 0.6 for men, >0.56 for women and asymptomatic
- Hb (upper limit of normal) with thrombosis or vascular concerns
Category 2 (appointment clinically indicated within 90 days)
- persistent unexplained elevated HCT, >0.52 for men and >0.48 for women or Hb > upper limit of normal, and JAK2 V617F or exon 12 mutation
Category 3 (appointment clinically indicated within 365 days)
- HCT persistently (3 months apart) above 0.52 to 0.60 in men and 0.48 to 0.56 in women and asymptomatic
Essential referral information
Completion required before first appointment to ensure patients are ready for care. Please indicate in the referral if the patient is unable to access mandatory tests or investigations as they incur a cost or are unavailable locally.
- past medical history
- medication history
- current medication list including supplements
- blood results:
- complete blood examination (CBE)
- blood film examination
- iron studies
- JAK2 V617F
- history of chronic hypoxia, smoking, renal disease, for example renal cysts, renal artery stenosis
Additional information to assist triage categorisation
- erythropoietin level, note that this test cannot be rebated under medicare, please check with pathology provider
- JAK2 exon 12 mutation testing, non medicare rebatable
- chest radiograph if respiratory issues
- ultrasound abdomen, renal or hepatic tumour
- testosterone replacement
Clinical management advice
Polycythaemia refers to an increase in haemoglobin above the normal range. This can be primary polycythaemia where there is an increase in red cell mass due to a mutation in red blood progenitor cells; or secondary polycythaemia where various conditions which lead to an increased erythropoietin production (such as hypoxia) can also cause an increase in haemoglobin. Certain medications for example anabolic steroids or androgens may also cause polycythaemia. Most cases are secondary polycythaemia.
Patients with secondary polycythaemia rarely benefit from venesection. It is important to rule out other non-haematological disorders such as renal cell carcinoma, hypoxia secondary to untreated obstructive sleep apnoea (OSA) or advanced pulmonary disease (COPD) which may cause secondary polycythaemia.
Do not give iron therapy to patients who are iron deficient but have an elevated haematocrit.
Do not give iron replacement for patients who have been receiving venesections for polycythaemia.
Clinical resources
- Leukaemia Foundation - Polycythaemia (Rubra) Vera
- Leukemia & Lymphoma Society - Polycythaemia Vera (PV)
- The Royal College of Pathologists of Australasia (RCPA) – Polycythaemia
- The Royal College of Pathologists of Australasia (RCPA) - Polycythaemia Vera
Consumer resources
Reason for request
- to establish a diagnosis
- for treatment or intervention
- for advice and management
- for specialist to take over management
- for a specified test/investigation the General Practitioner cannot order
- for other reason (e.g. rapidly accelerating disease progression)
- transfer of care from another tertiary service
- clinical judgement indicates a referral for specialist review is necessary.
Patient demographic details
- full name, including aliases
- date of birth
- residential and postal address
- telephone contact number/s – home, mobile and alternative
- Medicare number, where eligible
- name of the parent or caregiver, if appropriate
- preferred language and interpreter requirements
- identifies as Aboriginal and/or Torres Strait Islander
Clinical modifiers
- impact on employment
- impact on education
- impact on home
- impact on activities of daily living
- impact on ability to care for others
- impact on personal frailty or safety
- identifies as Aboriginal and/or Torres Strait Islander
Other relevant information
- Willingness to have surgery, where surgery is a likely intervention.
- Choice to be treated as a public or private patient.
- Compensable status, e.g. DVA, Work Cover, Motor Vehicle Insurance, etc.
- Relevant social history, including identifying if you feel your patient is from a vulnerable population, under guardianship/out-of-home care arrangements and/or requires a third party to receive correspondence on their behalf.
- Triage of a specialist outpatient referral is based on clinical decision making to allocate an appropriate urgency categorisation.
- Where appropriate and where available, the referral may be streamed to an associated public allied health and/or nursing service. Access to some specific services may include initial assessment and management by associated public allied health and/or nursing, which may either facilitate or negate the need to see the public medical specialist.
- A change in patient circumstance (such as condition deteriorating or pregnancy) may affect the urgency categorisation and should be communicated as soon as possible.
- All new referrals will be triaged by a consultant and appointment times scheduled according to clinical urgency.