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.

  • acute anaphylaxis
    • difficult/noisy breathing
    • swelling of tongue
    • swelling/tightness in throat
    • difficulty talking and/or hoarse voice, wheeze, or persistent cough
    • persistent dizziness or collapse
    • persistent abdominal pain, vomiting after insect sting
  • adrenaline has been administered

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

Southern Adelaide Local Health Network

Exclusions

  • patients with a diagnosis of Postural Orthostatic Tachycardia Syndrome (POTS) and/or Ehlers-Danlos syndrome who have normal serum tryptase and who do not have symptoms consistent with mast cell activation
  • patients who have vague or non-specific symptoms but no characteristic mast cell-related symptoms and normal serum tryptase
  • suspected or proven mast cell leukaemia, refer to haematology

Triage categories

Category 1 (appointment clinically indicated within 30 days)

  • primary mastocytosis with active symptoms
    • elevated baseline mast cell tryptase level (greater than 12.0 ng/mL) and
    • positive c-kit mutation (peripheral blood or bone marrow) and/or
    • biopsy proven cutaneous mastocytosis and/or
    • one or more of the accompanying mast-cell related symptoms:
  • skin: flushing, pruritus, urticaria, angioedema
  • cardiovascular: lightheadedness, collapse (hypotension)
  • respiratory: wheezing, stridor (bronchospasm, laryngeal oedema)
  • gastrointestinal: diarrhea, abdominal pain

Category 2 (appointment clinically indicated within 90 days)

  • baseline elevation in tryptase (greater than 12.0ng/mL) without clinical evidence of primary mastocytosis or active symptoms
  • cutaneous mastocytosis, for investigation of possible systemic involvement

Category 3 (appointment clinically indicated within 365 days)

  • nil

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.

Mast cell disorders is a heterogenous group of disorders presenting with episodic symptoms involving multiple systems that are attributable to mast cell mediator release (e.g. flushing, pruritus, wheeze, gastrointestinal symptoms). The diagnosis of a mast cell activation syndrome is based on consensus criteria which requires clinical assessment in conjunction with laboratory assessments, of which tryptase is the most accessible and validated test within Australia and New Zealand.

Clinical management advice

Identification and avoidance of triggers (e.g. allergens, physical)

This can be facilitated by Immunological assessment to confirm history and to facilitate further investigations if indicated e.g. skin prick testing, specific IgE testing if IgE-mediated reaction suspected.

Pharmacological management targeting mast cell mediators

  • medications that can be utilised in a stepwise fashion can include:
    • H1 histamine receptor antagonists e.g. cetirizine, desloratadine, fexofenadine, bilastine – non-sedating second generation agents preferred
    • H2 histamine receptor antagonists, e.g. nizatidine, famotidine
    • anti-leukotriene medications (montelukast)
    • mast cell stabilisers e.g. sodium cromoglycate, ketotifen
  • adrenaline autoinjector, if indicated

Clinical resources

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.