All information is correct at time of printing and is subject to change without notice. The Devon Formulary and Referral Website is not in any way liable for the accuracy of any information printed and stored by users. For the most up-to-date information, please refer to the website.
Referral
Mastocytosis (Adults)
Key Messages
- For efficiency and to minimise returned referrals, use of the referral form is recommended.
- Referrals not on the referral form will be accepted as long as all the essential information is included in a referral letter.
- See here to find the latest information for GPs regarding Immunology and Allergy
Scope
Adults (16 years or over) with:
- symptoms suggestive of mastocytosis AND a raised MCT
Out of Scope
Suspected Mast Cell Activation Syndrome (MCAS) will be assessed only if the patient meets the Vienna criteria for mastocytosis outlined here.
- See the following pathways for other allergic or atopic conditions:
Introduction
Mast cells are a type of white blood cell which are produced in the bone marrow and are present throughout the body. It is mast cells which are activated and release mediators (including histamine and tryptase) during anaphylaxis. Mastocytosis is a rare condition that involves an increase in number and/or activity of mast cells, which can predispose to more severe anaphylaxis.
The causes of anaphylaxis in people with mastocytosis are varied, but include stinging insect venom, medications, foods, and often non-allergic triggers such as temperature, exercise, stress, or alcohol, or in many cases the cause remains unknown.
In addition to anaphylaxis, people with mastocytosis may have a scattered pigmented rash (“urticaria pigmentosa”), gastrointestinal, musculoskeletal, or neurocognitive symptoms.
A history of episodes of the below symptoms may suggest a diagnosis of mastocytosis:
- flushing, itching or swelling
- abdominal pain or diarrhoea
- palpitations or shortness of breath
- severe anaphylaxis or collapse
A baseline Mast Cell Tryptase (MCT) level should be measured (expected to be raised in people with mastocytosis)
- Anaphylaxis should be treated immediately according to Resuscitation Council guidelines and the patient referred to hospital (see anaphylaxis guidance)
- oral non-sedating antihistamines up to 4x/day may be beneficial for symptoms of itching, rash, swelling as per the Chronic Urticaria/angioedema guideline.
The information essential for clinical triage is outlined on the referral form.
For efficiency and to minimise returned referrals, use of the referral form is recommended.
Referrals not on the referral form will be accepted as long as all the essential information is included in a referral letter.
Referral Criteria
Adults (16 years or over) with:
- symptoms suggestive of mastocytosis AND a raised MCT
Referral Instructions
e-Referral Service Selection
Specialty: Allergy
Clinic Type: Allergy
Service: DRSS-Eastern-Allergy-Devon ICB- 15N
Referral form
Pathway Group
This guideline has been signed off on behalf of NHS Devon.
Publication date: December 2024
