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
- Aggressive systemic Mastocytosis (ASM)
- Systemic Mastocytosis with Associated Hematologic Neoplasm (SM-AHN)
- 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 blasts2,5,6.
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.
Novartis would like to acknowledge the invaluable contribution of Leukemia Care in the development of this educational material.
References
1. Valent et al. Blood. Mastocytosis 2016: updated WHO classification and novel emerging treatment concepts. 2017 Mar 16; 129(11): 1420–1427.
2. Pardanani A. Am J Hematol. 2015;90:251-262.
3. National Health Service. Overview- Mastocytosis. https://www.nhs.uk/conditions/mastocytosis/. Accessed September 20, 2019
4. OncLive. Subtypes and Characteristics of Mastocytosis. https://www.onclive.com/insights-archive/aml-asm-advancements/subtypes-a.... Accessed September 23, 2019.
5. Cancer.net. Mastocytosis: Treatment Options. https://www.cancer.net/cancer-types/mastocytosis/treatment-options. Accessed September 23, 2019.
6. The Mastocytosis Society. Overview and diagnosis. https://tmsforacure.org/overview/. Accessed September 23, 2019.
7. Horny H. Am J Clin Pathol. Mastocytosis: An Unusual Clonal Disorder of Bone Marrow–Derived Hematopoietic Progenitor Cells. 2009;132:438-447.