The Facts on Advanced Systemic Mastocytosis

What is Advanced Systemic Mastocytosis (AdvSM)?

Advanced Systemic Mastocytosis is a group of rare mast cell disorders where the disease has spread to more than one part of your body, and is stopping your body from working¹.

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Types of Advanced Systemic Mastocytosis

If you have been told you have Advanced Systemic Mastocytosis, it is likely you will have one of these three types² 

1. Aggressive systemic Mastocytosis (ASM)
2. Systemic Mastocytosis with Associated Hematologic Neoplasm (SM-AHN)
3. Mantle Cell Leukaemia (MCL)

There is no cure for Advanced Systemic Mastocytosis, but there are treatments that can help³.

Find out more about Systemic Mastocytosis and the types

Symptoms

Some symptoms of AdvSM may include:

• Losing weight without trying²
• Fever or chills⁴
• Bone pain⁴
• Swollen lymph nodes, liver or spleen²
• Tiny red spots on your skin⁴

Other symptoms may include:

Poor appetite, pain when you go to the toilet, anaemia, stomach problems such as feeling sick, being sick or the runs, anaphylactic shock, low blood pressure, low platelet count, low levels of albumin and too many bone marrow blasts^2,5,6.

Tests for AdvSM

Tests for AdvSM may include a combination of⁴:

• Full blood count and blood tryptase levels
• Bone marrow test (biopsy)
• Ultrasound scan
• DEXA scan (bone scan)

Other tests may include⁷:

Samples taken from areas such as your spleen or lymph nodes to check for the number or shape of mast cells in other parts of the body.

Questions to ask your doctor

• What type of mastocytosis do I have?
• Is my mastocytosis a type of cancer?
• Should I carry an EpiPen?
• How will advanced SM affect me?
• What things can I no longer do?
• What are my treatment options?
• Are there any risks to the treatment?
• What are the side effects of the treatment?
• Will my symptoms go away after I start treatment?
• How will I know if my treatment is working?
• If treatment is not helping, what are my options?
• Should I take part in a clinical trial?
• How long do people with this disease live?
• Are there support groups I can join?

Treatments for AdvSM

There is no cure for AdvSM but there are treatments available⁵.

There are two types of treatment approaches for AdvSM:

• Treatments to treat the symptoms, also known as supportive treatments. These reduce the symptoms that can cause quality of life issues.
• Treatments to stop the mast cells from growing and spreading. When mast cells grow too fast, they can stop some of the body’s organs from working properly. Although treatment can’t reduce the number of mast cells in your body, the treatments can try to stop them spreading.