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MASTOSYTOSIS
PART A
Mastocytosis is a rare condition caused by an excess number of mast cells gathering in
the body's tissues. There are 2 main types of mastocytosis:

• cutaneous mastocytosis, which mainly affects children – where mast cells gather
in the skin, but are not found in large numbers elsewhere in the body
• systemic mastocytosis, which mainly affects adults – where mast cells gather in
body tissues, such as the skin, internal organs and bones
There are also several subtypes of systemic mastocytosis, depending on the symptoms.

Mast cells are produced in the bone marrow, the spongy tissue found in the hollow
centres of some bones, and live longer than normal cells. They're an important part
of the immune system and help fight infection. When mast cells detect a substance that
triggers an allergic reaction (an allergen), they release histamine and other
chemicals into the bloodstream. Histamine makes the blood vessels expand and the
surrounding skin itchy and swollen. It can also create a build-up of mucus in the
airways, which become narrower. The symptoms of mastocytosis can vary depending
on the type.

Cutaneous mastocytosis: The most common symptom of cutaneous mastocytosis is

abnormal growths (lesions) on the skin, such as bumps and spots, which can form on
the body and sometimes blister.

Systemic mastocytosis: Some people with systemic mastocytosis may experience

episodes of severe symptoms that last 15-30 minutes, often with specific triggers such
as physical exertion or stress. Many people do not have any problems.

PART B

There are 3 subtypes of systemic mastocytosis. They are:

• indolent mastocytosis – symptoms are usually mild to moderate and vary from
person to person; indolent mastocytosis accounts for around 90% of adult
systemic mastocytosis cases
• aggressive mastocytosis – where mast cells multiply in organs, such as the
spleen, liver and digestive system; the symptoms are more wide-ranging and
severe, although skin lesions are less common
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• systemic mastocytosis with associated blood (haematological) disease – where a

condition that affects the blood cells, such as chronic leukaemia, also develops
Severe allergic reaction

People with mastocytosis have an increased risk of developing a severe and life-
threatening allergic reaction. This is known as anaphylaxis .The increased risk of
anaphylaxis is caused by the abnormally high number of mast cells and their potential
to release large amounts of histamine into the blood .If you or your child has
mastocytosis, you may need to carry an adrenaline auto-injector, which can be used to
treat the symptoms of anaphylaxis.

The cause or causes of mastocytosis are not fully known, but there's thought to be an
association with a change in genes known as the KIT mutation .The KIT mutation
makes the mast cells more sensitive to the effects of a signalling protein called stem cell
factor (SCF) .SCF plays an important role in stimulating the production and survival of
certain cells, such as blood cells and mast cells, inside the bone marrow .In a very few
cases of mastocytosis it appears the KIT mutation is passed down through families.
However, in most cases the mutation happens for no apparent reason.
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PART C
MAST CELL ACTIVATION

PART D
The treatment options for mastocytosis depend on which type you have and how
severe your symptoms are. Nearly all medicines used to treat mastocytosis are
unlicensed. In other words, the medicine has not undergone clinical trials to see if it can
treat mastocytosis effectively and safe.

Steroid cream: Mild to moderate cases of cutaneous mastocytosis can be treated with
a very strong steroid cream (topical corticosteroids) for a limited length of time, usually
up to 6 weeks. Steroid cream reduces the number of mast cells that can release histamine
and trigger inflammation inside the skin
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Antihistamines: Antihistamines can also be used to treat symptoms of cutaneous or

indolent mastocytosis, such as itchiness and skin redness. Antihistamines are a type of
medicine that blocks the effects of histamine. They're widely used to treat allergic
conditions.

Sodium cromoglicate: Sodium cromoglicate is a medicine used to

treat conjunctivitis, asthma and food allergy. It may also be used to treat gut
symptoms of mastocytosis, but is not absorbed well from the bowel. Sodium
cromoglicate 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, bone pain,
fatigue and headache. Some people taking sodium cromoglicate have reported feeling
sick and getting joint pain.

PUVA: More severe symptoms of cutaneous mastocytosis, such as severe itchy

skin, may require a type of treatment called psoralen plus ultraviolet A (PUVA).
PUVA involves taking a medicine 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 reduce lesions in the skin.

Steroid tablets: If symptoms such as itchiness are particularly severe, corticosteroid

tablets (oral corticosteroids) may be prescribed on a short-term basis. However, this is
rare. A short course of corticosteroid tablets may be recommended if you have bone
pain caused by mastocytosis, or a severe allergic reaction (anaphylaxis).

Questions 1-7 For each question, 1-7, decide which text (A, B, C or D) the
Information comes from. You may use any letter more than once. In which text
can you can find information about.

1. What are the neuropsychiatric symptoms of mast cell activation?

2. What type of medicines are used to treat allergic reaction?

3. Sub divisions of mastosytosis?

4. Which is the most typical symptom of cutaneous mastocytosis?

5. What is known as anaphylaxis?

6. Are the medications are used to treat mastosytosis are authorised?

7. PUVA stands for?

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Answer the questions,8-14, with a word or short phrase from the texts.
Each answer may include words, numbers or both.
8. Which tablets are recommended for a short-term basis in the treatment of mastocytosis?

9. What are the symptoms of mast cell activation in oropharymgeal?

10. What makes the mast cells more sensitive to the effects of signalling protein?

11. What is used to treat symptoms of anaphylaxis in child?

12. Which cells are live longer than normal cells?

13. People with systemic mastocytosis may experience?

14. Which medicine that blocks the effects of histamine?

Questions 15-20. Complete each of the sentences, 15-20, with a word or

short phrase from one of the texts. Each answer may include words,
numbers or both.
15. When mast cells detect a substance that cause an allergic reaction, they
release.................and other chemicals?

16. .....................reduces the number of mast cells that can produce histamine and
inflammation inside the skin?

17. ..........................are an important part of the immune system and fight to

infection?

18. An association with a change in genes known as the..........................?

19. ........................symptoms are usually mild to moderate and vary from person to
person?

20. Histamine makes mucus in the airways which become..........................?

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ANSWER KEY
1. C

2. D

3. B

4. A

5. B

6. D

7. D

8. ORAL CORTICOSTEROIDS

9. BURNING PAIN, APHTHAE

10. THE KIT MUTATION

11. ADRENALIN AUTO-INJECTOR

12. MAST CELLS

13. EPISODES OF SEVERE SYMPTOMS

14. ANTIHISTAMINES

15. HISTAMINE

16. STEROID CREAM

17. MAST CELLS

18. KIT MUTATION

19. INDOLENT MASTOCYTOSIS

20. NARROWER