Mastocytosis - Treatment 

Treating mastocytosis 

Unlicensed treatments

Many medications mentioned on this page are unlicensed for the treatment of mastocytosis.

This means the manufacturers of the medications have not applied for a license for their medication to be used in treating mastocytosis. In other words, the medication has not undergone clinical trials to see if it can treat mastocytosis effectively and safely.

Many experts will use an unlicensed medication if they think it likely to be effective and the benefits of treatment outweigh any associated risk.

The treatment options for mastocytosis depend on which type you have, and how severe your symptoms are.

Steroid cream

Mild to moderate cases of cutaneous mastocytosis can be treated with steroid cream (topical corticosteroids). Steroid cream prevents mast cells releasing histamine and triggering inflammation inside the skin.

Side effects of steroid cream include:

  • thinning of the skin, which can sometimes result in permanent stretch marks
  • discolouration of the skin
  • the affected area of skin bruising easily

To reduce the risk of side effects, you should only apply the cream to areas of skin affected by lesions.

Antihistamines

Antihistamines may also be used to treat symptoms of cutaneous or indolent mastocytosis, such as itchiness and redness of the skin. Antihistamines are a type of medication that block the effects of histamine. They are widely used in the treatment of allergic conditions.

Side effects of antihistamines include:

  • headache
  • dry mouth
  • dry nose

These side effects should pass quickly.

Sodium cromoglicate

Sodium cromoglicate (Nalcrom) is a medication used to treat allergic conditions of the eye, rhinitis and food allergy. It is also used as an unlicensed medication for the treatment of mastocytosis.

It is a mast cell stabiliser, which means it reduces the amount of chemicals released by the mast cells. This helps relieve symptoms such as diarrhoea, itching and flushing of the skin.

case report published in 2010 describes a patient with mastocytosis whose symptoms improved after taking antihistamines and sodium cromoglicate capsules. Their bone pain, fatigue and headache improved further after also taking inhaled sodium cromoglicate.

Nausea, skin rashes and joint pains have been reported in some people taking sodium cromoglicate. A topical form (applied to the skin) is available which may help with itching.

PUVA

The more severe symptoms of cutaneous mastocytosis, such as severe itchy skin, may require a type of treatment known as psoralen plus ultraviolet A (PUVA). PUVA involves taking a medication called psoralen, which makes the skin more sensitive to the effects of ultraviolet light.

The skin is then exposed to a wavelength of light called ultraviolet A (UVA), which helps remove lesions from the skin.

The patient can only receive a limited number of PUVA sessions as using the treatment too many times (thought to be around 200-250 sessions) increases their risk of developing skin cancer.

Steroid tablets

During times when symptoms such as itchiness are particularly severe, tablets containing corticosteroids (oral corticosteroids) may be prescribed on a short-term basis.

For example, a short course of corticosteroid tablets may be recommended if you have bone pain or a previous history of anaphylaxis (severe allergic reaction).

Side effects of oral corticosteroids used on a short-term basis include:

  • an increase in appetite
  • weight gain
  • insomnia
  • fluid retention
  • mood changes, such as feeling irritable or anxious

Bisphosphonates and calcium supplements

If you have osteoporosis (weakened bones) due to abnormal mast activity in your bones, you will be given a type of medication called bisphosphonates. Bisphosphonates slow the process of bone breakdown while allowing production of new bone to continue as normal, which improves your bone density.

You may also be given calcium supplements (calcium helps strengthen the bones).

Read more information about treating osteoporosis.

H2-receptor antagonists

If you have symptoms of stomach pain due to peptic ulcers, you will be given a medication called a H2-receptor antagonist. This blocks the effects of histamine in the stomach (histamine stimulates the production of stomach acid, which damages the lining of the stomach). 

Read more information about treating a peptic ulcer.

Interferon alpha

Originally designed to treat cancer, interferon alpha has proved effective in treating some cases of aggressive mastocytosis. Exactly why this is the case is uncertain, although it appears the medication reduces the production of mast cells inside the bone marrow.

Interferon alpha is given by injection. Most people will have flu-like symptoms such as chills, a high temperature and joint pain when they first start taking interferon alpha. However symptoms should improve over time as your body gets used to the medicine.

Nilotinib and dasatinib

Nilotinib or dasatinib may be tried when people do not respond to treatment with imatinib. They work in much the same way, blocking the effects of tyrosine kinase.

The medication will make you more vulnerable to infection, so report possible symptoms of infection to your GP immediately.

Cladribine

Cladribine was originally designed to treat leukaemia (cancer of the white blood cells), but has since proved effective in treating aggressive systematic mastocytosis.

Cladribine suppresses the activity of your immune system. It is given by infusion, which means it is slowly released into your body via a drip in your arm over the course of two hours.

Again, cladribine will make you more vulnerable to the effects of infection, so you should report possible symptoms of infection to your GP immediately.

Imatinib

An alternative medication to interferon alpha is imatinib tablets. Imatinib blocks the effects of an enzyme called tyrosine kinase, which helps stimulate  production of mast cells.

However, imatinib should only be used for people who do not have the c-KIT mutation (see Mastocytosis  causes), and it does not work for all cases of mastocytosis.

Imatinib can also make you more vulnerable to infection. Contact your GP immediately if you develop possible signs of an infection, such as:

  • high temperature (fever) of or above 38ºC (101.4ºF)
  • headache
  • aching muscles
  • diarrhoea
  • tiredness

Treatments for haematological (blood) disease

Systemic mastocytosis with associated haematological disease will be treated in the same way as aggressive systematic mastocytosis (with either interferon alpha, imatinib, nilotinib or cladribine) with a number of additional treatments for the related haematological condition.

For more information on treating the most common haematological conditions, see:

Myeloproliferative neoplasms are also haematological disorders that can be associated with mastocytosis.

Using an adrenaline injection pen

Because of your increased risk of anaphylaxis, you may be given an adrenaline injection pen to use in an emergency. Adrenaline is a hormone that helps block the effects of histamine while also relieving breathing difficulties. Each pen contains a single dose of adrenaline (0.3mg for adults or 0.15mg for children). There are two types:

  • EpiPen this needle releases adrenaline when jabbed against the outer thigh
  • Anapen you hold the syringe against the outer thigh and push a button, which plunges the adrenalin-loaded needle into your thigh. After injecting, the syringe should be held in place for 10 seconds

The injections can be given through clothing.

If you are given an adrenaline injection pen it is important to keep an eye on its expiry date as it will not be effective beyond this date.


Page last reviewed: 28/06/2012

Next review due: 28/06/2014

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Antihistamines

Antihistamines are a type of medicine that are often used to treat allergic health conditions