Mastocytosis and Mast Cell Disorders - Treatment and Prognosis

How are mastocytosis and other mast cell disorders treated?

There is no treatment that will actually cure these conditions but a wide variety of medicines is available to control the symptoms.

Acute anaphylaxis

If you're prone to sudden severe allergy symptoms you should avoid trigger factors. It's a good idea to wear a medical emergency identification bracelet or similar. If you've been given treatment advice from a specialist about what to do during an attack, carry it with you. Acute severe reactions are usually treated with adrenaline (epinephrine) injections, anti-allergy medication, fluids into a vein and steroids. You might need to carry an adrenaline (epinephrine) pen injector with you which can be obtained from your doctor. Some people need a course of injections to reduce their sensitivity to insect stings.

Skin and symptoms related to blood circulation

  • Itching, flushing and other skin allergy symptoms can be controlled by medicines called H1-receptor and H2-receptor antagonists. These include chlorphenamine, ketotifen and cimetidine.
  • Medication which stabilises the mast cells such as sodium cromoglicate, nedocromil and ketotifen.
  • Steroid creams or ointments and injections are sometimes used.
  • Treatment also used for psoriasis - called psoralen combined with ultraviolet A (PUVA) treatment - sometimes helps.

Wheezing

Inhalers to relax the airways - for example, salbutamol - can be helpful.

Digestive symptoms

  • H2-receptor antagonists or proton pump inhibitors help to control the effects of excess acid.
  • Oral sodium cromoglicate is beneficial in people with diarrhoea and tummy (abdominal) pain.
  • Anticholinergic medication such as propantheline (which blocks the effects of a body chemical called acetylcholine) may help control diarrhoea.

Other treatments

Medicines called leukotriene inhibitors used mainly in asthma - for example, montelukast - have been used. Low-dose aspirin and steroid tablets have been found helpful to control many different symptoms.

If the condition is severe, surgery to remove the spleen (splenectomy), medicines that have an effect on the immune system (such as interferon), bone marrow transplant or stem cell therapy may be considered.

What is the outlook for these conditions?

Cutaneous mastocytosis in children often settles down with time. Adults can go on to develop the systemic form.

Systemic mastocytosis tends to be persistent and the outlook (prognosis) depends on which parts of the body are involved. In most cases, it just keeps going at a low level for decades, requiring symptomatic treatment. Occasionally, however, it can become aggressive and even life-threatening.

Most cases of localised mastocytosis cause lumps that are nothing to worry about but occasionally cancers do occur which invade the surrounding tissue.

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  • Cardet JC, Akin C, Lee MJ; Mastocytosis: update on pharmacotherapy and future directions. Expert Opin Pharmacother. 2013 Oct 14(15):2033-45. doi: 10.1517/14656566.2013.824424.
  • Maculopapular cutaneous mastocytosis; DermNet NZ, 2014
  • Pardanani A; Systemic mastocytosis in adults: 2017 update on diagnosis, risk stratification and management. Am J Hematol. 2016 Nov 91(11):1146-1159. doi: 10.1002/ajh.24553.
Author:
Dr Laurence Knott
Peer Reviewer:
Dr John Cox
Document ID:
29438 (v1)
Last Checked:
09 July 2017
Next Review:
08 July 2020

Disclaimer: This article is for information only and should not be used for the diagnosis or treatment of medical conditions. Patient Platform Limited has used all reasonable care in compiling the information but make no warranty as to its accuracy. Consult a doctor or other health care professional for diagnosis and treatment of medical conditions. For details see our conditions.