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Mastocytosis - Overview

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2015

ON THIS PAGE: You will find some basic information about this disorder, which may or may not become cancerous, and the parts of the body it may affect. This is the first page of Cancer.Net’s Guide to Mastocytosis. To see other pages, use the menu on the side of your screen. Think of that menu as a roadmap to this full guide.

Mast cells are a type of blood cell made in the bone marrow. They are involved in allergic reactions and help fight off parasitic infections. Mast cells produce histamine, a chemical that can cause itching, sneezing, congestion, swelling, and wheezing.

About mastocytosis

Mastocytosis describes a group of disorders that are caused by too many mast cells in the body. The number of mast cells in the body can increase as a result of some non-cancerous (benign) or reactive conditions. However, certain types of cancers begin when healthy mast cells change and grow uncontrollably.

There are two general types of mastocytosis:

  • Cutaneous mastocytosis. This type of mastocytosis involves the skin.

  • Systemic mastocytosis. This type of mastocytosis involves the entire body.

Cutaneous mastocytosis

Cutaneous mastocytosis is an increase of mast cells in the skin. About 90% of people with mastocytosis have the cutaneous type. Subtypes of cutaneous mastocytosis include:

  • Urticaria pigmentosa. This is the most common type of cutaneous mastocytosis. The main sign of urticaria pigmentosa is the appearance of tan or red-brown spots on the skin. These spots usually develop on the midsection of the body first and then spread over the rest of the body. A person with this type of mastocytosis may also experience nausea, vomiting, and diarrhea.

  • Solitary mastocytoma. This type of mastocytosis is more common in infants and children than in adults. It usually causes a large nodule, about 3 centimeters (cm) to 4 cm in diameter, to form on an arm or leg. This nodule is called a mastocytoma.

  • Diffuse erythrodermic mastocytosis. This type of mastocytosis is found most often in children younger than three years old. Diffuse erythrodermic mastocytosis may not be noticeable when a child is born, but it can later show up as a rapid thickening of the skin. Symptoms of systemic mastocytosis (see the Symptoms and Signs section) and blisters are also common.

  • Telangiectasia macularis eruptiva perstans. This type of cutaneous mastocytosis develops mainly in adults. The most common sign is lesions that do not itch and are smaller than urticaria pigmentosa.

Systemic mastocytosis

Systemic mastocytosis involves internal organs throughout the body, including the gastrointestinal tract, bone marrow, liver, spleen, and lymph nodes. Lymph nodes are tiny, bean-shaped organs that help fight infection. About 85% of the time, urticaria pigmentosa (see above) develops first.

Depending on the number of mast cells in an organ, the disease is classified as either indolent, which means it is slow-growing, or aggressive, which means it is fast-growing. As the number of mast cells builds up in an organ, the symptoms of the disease may get worse.

Systemic mastocytosis can become cancerous. The risk of systemic mastocytosis becoming cancerous is 7% when the disease begins in childhood and as much as 30% in adults. Mast cell leukemia involves the blood, while mast cell sarcoma involves the body’s soft tissues.

The next section in this guide is Statistics, and it helps explain how many people are diagnosed with this condition. Or, use the menu on the side of your screen to choose another section to continue reading this guide.  

Mastocytosis - Statistics

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2015

ON THIS PAGE: You will find information about how many people are diagnosed with this disorder each year. To see other pages, use the menu on the side of your screen.

Mastocytosis is a rare disorder, and its true incidence rates are unknown.

The next section in this guide is Risk Factors and it explains what factors may increase the chance of developing this disease. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Mastocytosis - Risk Factors

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2015

ON THIS PAGE: You will find out more about the factors that increase the chance of developing this condition. To see other pages, use the menu on the side of your screen.

A risk factor is anything that increases a person’s chance of developing a disease. Although risk factors often influence the development of mastocytosis, most do not directly cause the condition. Some people with several risk factors never develop mastocytosis, while others with no known risk factors do. However, knowing your risk factors and talking about them with your doctor may help you make more informed lifestyle and health care choices.

The following factors may raise a person’s risk of developing mastocytosis:

  • Age. Most often, cutaneous mastocytosis begins during infancy and early childhood. The risk of developing systemic mastocytosis increases with age.

  • c-kit receptor mutation. The c-kit receptor is a protein on the surface of some cells that binds to a substance that causes them to grow. Changed or mutated forms of the c-kit gene may cause some types of mastocytosis, including systemic mastocytosis.

The next section in this guide is Symptoms and Signs, and it explains what body changes or medical problems this condition can cause. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Mastocytosis - Symptoms and Signs

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2015

ON THIS PAGE: You will find out more about body changes and other things that can signal a problem that may need medical care. To see other pages, use the menu on the side of your screen.

People with mastocytosis may experience the following symptoms or signs. Sometimes, people with mastocytosis do not show any of these symptoms. Or, these symptoms may be caused by another medical condition.

General symptoms

  • Hives

  • Red, itchy rash

  • Diarrhea

  • Abdominal pain

  • Fainting

  • Facial flushing or reddening of the face

  • Shortness of breath

  • Wheezing or trouble breathing

  • Psychological changes, for example, irritability or difficulty concentrating

Symptoms of urticaria pigmentosa

  • Tan or red-brown spots on the skin

  • Abdominal pain

  • Nausea

  • Vomiting

  • Diarrhea

  • Headache

  • Increased heart rate

  • Facial flushing

  • Psychological changes

Symptoms of solitary mastocytoma

  • Raised or flat reddish-brown spot on the skin

  • Hives

  • Itching

Symptoms of diffuse erythrodermic mastocytosis

  • Thickening of the skin

  • Blisters

Symptom of telangiectasia macularis eruptiva perstans

  • Small lesions that do not itch

Symptoms of systemic mastocytosis

  • Skin lesions

  • Urticaria pigmentosa

  • Facial flushing

  • Itching

  • Nausea

  • Vomiting

  • Diarrhea

  • Abdominal pain

  • Ulcers in the stomach and duodenum (small intestine)

  • Headache

  • Lightheadedness

  • Heart palpitations, an irregular or unusually rapid beating of the heart

  • Bone pain

  • Anemia, a low red blood cell count, which can cause fatigue

  • Psychological changes

The symptoms of systemic mastocytosis can sometimes occur as “attacks,” where more than one symptom appears at the same time. Following an attack, the person may feel tired and lethargic.

If you are concerned about one or more of the symptoms or signs on this list, please talk with your doctor or a dermatologist, a doctor who specializes in skin conditions. The doctor will ask how long and how often you’ve been experiencing the symptom(s), in addition to other questions. This is to help find out the cause of the problem, called a diagnosis.

If mastocytosis is diagnosed, relieving symptoms is an important part of your care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your health care team about symptoms you experience, including any new symptoms or a change in symptoms.

The next section in this guide is Diagnosis, and it explains what tests may be needed to learn more about the cause of the symptoms. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Mastocytosis - Diagnosis

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2015

ON THIS PAGE: You will find a list of the common tests and procedures that doctors can use to find out what’s wrong and identify the cause of the problem. To see other pages, use the menu on the side of your screen.

Doctors use many tests to diagnose a disease and find out its extent. Some tests may also determine which treatments may be the most effective. A biopsy is the only way to make a definitive diagnosis of mastocytosis. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis.

This list describes options for diagnosing this condition, and not all tests listed will be used for every person. Your doctor may consider these factors when choosing a diagnostic test:

  • Age and medical condition

  • Type of disease suspected

  • Signs and symptoms

  • Previous test results

In addition to a physical examination, the following tests may be used to diagnose mastocytosis:

  • Blood/urine tests. Tests are done on samples of the patient’s blood and urine to help find high levels of mast cells and histamine. Blood tests can also show how well the liver, kidneys, and other organs are working. The blood’s level of an enzyme called tryptase may be higher than normal for people with systemic mastocytosis. However, a high level of tryptase may also occur as a result of other non-cancerous or reactive conditions.

  • Biopsy. Skin biopsies are often used to diagnose mastocytosis. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that mastocytosis is present, but only a biopsy can make a definite diagnosis. A pathologist then analyzes the sample(s) removed during the biopsy. A pathologist is a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease.

  • Bone marrow aspiration and biopsy. Bone marrow aspiration and biopsy are similar procedures and are often done at the same time to examine the bone marrow. Bone marrow has both a solid and a liquid part. A bone marrow aspiration removes a sample of the fluid with a needle. A bone marrow biopsy is the removal of a small amount of solid tissue using a needle. A pathologist then analyzes the sample(s) to help diagnose systemic mastocytosis.

    A common site for a bone marrow aspiration and biopsy is the pelvic bone, which is located in the lower back by the hip. The skin in that area is numbed with medication beforehand, and other types of anesthesia (medication to block the awareness of pain) may be used.

  • Molecular testing. Your doctor may recommend running laboratory tests on tissue samples to identify specific genes, proteins, and other factors unique to the mast cells. Results of these tests will help decide whether your treatment options include a type of treatment called targeted therapy.

After these diagnostic tests are done, your doctor will review all of the results with you. If the diagnosis is mastocytosis, these results will help the doctor identify the type and plan treatment.

The next section in this guide is Stages, and it explains the system doctors use to describe the extent of cancerous conditions. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Mastocytosis - Stages

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2015

ON THIS PAGE: You will learn that because mastocytosis is not a type of cancer there is no standard staging system. To see other pages, use the menu on the side of your screen.

Staging is a way of describing where a cancer is located, if or where it has spread, and whether it is affecting other parts of the body. Because mastocytosis is not a type of cancer, there is no standard staging system.

If systemic mastocytosis does become cancerous, the doctor will use diagnostic tests to find out the cancer's stage. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis, which is the chance of recovery. There are different stage descriptions for different types of cancer.

The next section in this guide is Treatment Options, and it helps explain the different treatments that may be used for mastocytosis. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Mastocytosis - Treatment Options

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2015

ON THIS PAGE: You will learn about the different ways doctors use to treat people with mastocytosis. To see other pages, use the menu on the side of your screen.

This section outlines treatments that are the standard of care (the best known treatments available) for the various types of mastocytosis. When making treatment plan decisions, patients are also encouraged to consider clinical trials as an option. A clinical trial is a research study to test a new approach to treatment to evaluate whether it is safe, effective, and possibly better than the standard treatment. Clinical trials may test such approaches as a new drug, a new combination of standard treatments, or new doses of current therapies. Your doctor can help you review all treatment options. For more information, visit the About Clinical Trials and Latest Research sections.

Treatment overview

The treatment of mastocytosis depends on the type, the symptoms of the disease, its extent, and the person’s overall health. In many cases, different types of doctors often work together to create a patient’s overall treatment plan that combines different types of treatments. This is called a multidisciplinary team. Medical teams also include a variety of other health care professionals, including physician assistants, nurses, social workers, pharmacists, counselors, dietitians, and others.

There is no cure for mastocytosis, although several treatments can be used to relieve symptoms and remove a mastocytoma (see the Overview section). Descriptions of the most common treatment options for mastocytosis are listed below. Take time to learn about all of your treatment options and be sure to ask questions about things that are unclear. Also, talk about the goals of each treatment with your doctor and what you can expect while receiving the treatment. Learn more about making treatment decisions.

Managing symptoms

An important part of treating mastocytosis is controlling a person’s symptoms. One important way to do this is to avoid anything that may cause mast cells to release histamine. This may include extreme temperatures, alcohol, emotional stress, insect bites, and certain medications. For example, nonsteroidal anti-inflammatory drugs (NSAIDs) should be used with caution, as these drugs may trigger the release of histamine and can cause severe reactions or an attack.

Doctors may also recommend the following treatments to help relieve mastocytosis symptoms.

  • Antihistamines. Antihistamines help relieve itching, flushing, and other skin reactions.

  • Steroids. Steroids may be used to reduce the size of skin lesions.

  • Epinephrine. People with mastocytosis often carry a syringe of epinephrine, a hormone made by the adrenal glands, in case of a severe allergic reaction.

  • Other medications. Other medications can help relieve diarrhea and stomach pain.

  • Ultraviolet light. Sometimes, exposing the skin rashes to a source of ultraviolet (UV) light can help relieve symptoms of cutaneous mastocytosis.

Surgery

Surgery may be performed to remove lesions, such as a mastocytoma. Many surgeries on the skin can be performed quickly and easily. Learn more about the basics of surgery.

Targeted therapy

Targeted therapy is a treatment that targets the disease’s specific genes, proteins, or the tissue environment unique to mastocytosis that contribute to its growth and survival. This type of treatment blocks the growth of mast cells while limiting damage to healthy cells.

Recent studies show that not all diseases have the same targets. To find the most effective treatment, your doctor may run tests to identify the genes, proteins, and other factors involved in your mastocytosis. This helps doctors better match each patient with the most effective treatment whenever possible. In addition, many research studies are taking place now to find out more about specific molecular targets and new treatments directed at them.

Tyrosine kinase inhibitors are a type of targeted therapy. For mastocytosis, the target is the unique protein called the c-kit tyrosine kinase receptor (see the Risk Factors section). Treatment with tyrosine kinase inhibitors, including dasatinib (Sprycel), midostaurin (PKC412, still being tested in clinical trials), and less commonly imatinib (Gleevec) and nilotinib (Tasigna), may be considered for patients with mast cells that have a mutation in the c-kit tyrosine kinase receptor. See the Latest Research section for more information. Patients with more advanced systemic mastocytosis should be tested for this genetic mutation. 

Talk with your doctor about possible side effects for a specific targeted therapy and how they can be managed. Learn more about the basics of targeted treatments.

Stem cell transplantation/bone marrow transplantation

A stem cell transplant is a medical procedure in which unhealthy bone marrow is replaced by highly specialized cells, called hematopoietic stem cells, that develop into healthy bone marrow. Hematopoietic stem cells are blood-forming cells found both in the bloodstream and in the bone marrow. Today, this procedure is more commonly called a stem cell transplant, rather than bone marrow transplant, because it is the stem cells in the blood that are typically being transplanted, not the actual bone marrow tissue.

There are two types of stem cell transplantation depending on the source of the replacement blood stem cells: allogeneic (ALLO) and autologous (AUTO). ALLO uses donated stem cells, while AUTO uses the patient’s own stem cells. In both types, the goal is to destroy all unhealthy cells in the marrow, blood, and other parts of the body using high doses of chemotherapy and/or radiation therapy and then allow replacement blood stem cells to create healthy bone marrow.

Stem cell transplantation is not used frequently as a treatment for mastocytosis because treatment results are inconsistent and there are significant risks associated with this treatment approach. Learn more about the basics of bone marrow and stem cell transplantation.

Chemotherapy for mastocytosis-related cancer

Chemotherapy is sometimes recommended if mastocytosis becomes cancerous. Chemotherapy is the use of drugs to destroy cancer cells, usually by stopping the cancer cells’ ability to grow and divide. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication, or a hematologist, a doctor who specializes in treating blood disorders.

Systemic chemotherapy gets into the bloodstream to reach cancer cells throughout the body. Common ways to give chemotherapy include an intravenous (IV) tube placed into a vein using a needle or in a pill or capsule that is swallowed (orally).

A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a set period of time. A patient may receive one drug at a time or combinations of different drugs at the same time.

The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, hair loss, loss of appetite, and diarrhea. These side effects usually go away once treatment is finished.

Learn more about the basics of chemotherapy and preparing for treatment. The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions by using searchable drug databases.

Getting care for side effects

Mastocytosis treatment often causes side effects. In addition to treatment to manage the condition, an important part of treatment is relieving these side effects. This approach is called palliative or supportive care, and it includes supporting the patient with his or her physical, emotional, and social needs.

Palliative care is any treatment that focuses on reducing symptoms (see section above) and treatment side effects, improving quality of life, and supporting patients and their families. Any person, regardless of age or type of disease, may receive palliative care. It works best when palliative care is started as early as needed in the treatment process.

People often receive treatment for the disease and treatment to ease side effects at the same time. In fact, patients who receive both often have less severe symptoms, better quality of life, and report they are more satisfied with treatment.

Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, emotional support, and other therapies. Talk with your doctor about the goals of each treatment in your treatment plan.

Before treatment begins, talk with your health care team about the possible side effects of your specific treatment plan and palliative care options. And during and after treatment, be sure to tell your doctor or another health care team member if you are experiencing a problem so it can be addressed as quickly as possible. Learn more about palliative care.

The next section in this guide is About Clinical Trials, and it offers more information about research studies that are focused on finding better ways to care for people with mastocytosis. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Mastocytosis - About Clinical Trials

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2015

ON THIS PAGE: You will learn more about clinical trials, which are the main way that new medical approaches are tested to see how well they work. To see other pages, use the menu on the side of your screen.

What are clinical trials?

Doctors and scientists are always looking for better ways to care for patients with mastocytosis. To make scientific advances, doctors create research studies involving volunteers, called clinical trials. In fact, every drug that is now approved by the U.S. Food and Drug Administration (FDA) was previously tested in clinical trials.

Many clinical trials are focused on new treatments, evaluating whether a new treatment is safe, effective, and possibly better than the current (standard) treatment. These types of studies evaluate new drugs, different combinations of existing treatments, new approaches to surgery, and new methods of treatment. Patients who participate in clinical trials are often among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment.

There are also clinical trials that study new ways to ease symptoms and side effects during treatment and manage the late effects that may occur after treatment. Talk with your doctor about clinical trials regarding side effects. In addition, there are ongoing studies about ways to prevent the disease.

Deciding to join a clinical trial

Patients decide to participate in clinical trials for many reasons. For some patients, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other patients volunteer for clinical trials because they know that these studies are the only way to make progress in treating mastocytosis. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with mastocytosis.

Sometimes people have concerns that, in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill.” When a placebo is used in a study, it is done with the full knowledge of the participants. Find out more about placebos in cancer clinical trials.

Patient safety and informed consent

To join a clinical trial, patients must participate in a process known as informed consent. During informed consent, the doctor should list all of the patient’s options, so that the person understands how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different from the risks of standard treatment. Finally, the doctor must explain what will be required of each patient in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment.

Patients who participate in a clinical trial may stop participating at any time for any personal or medical reason. This may include that the new treatment is not working or there are serious side effects. Clinical trials are also closely monitored by experts who watch for any problems with each study. It is important that patients participating in a clinical trial talk with their doctor and researchers about who will be providing their treatment and care during the clinical trial, after the clinical trial ends, and/or if the patient chooses to leave the clinical trial before it ends. 

Finding a clinical trial

Research through clinical trials is ongoing for all types of cancer. For specific topics being studied for mastocytosis, learn more in the Latest Research section.

Cancer.Net offers a lot of information about clinical trials in other areas of the website, including a complete section on clinical trials and places to search for clinical trials for a specific type of cancer or cancer-related condition.

In addition, this website offers free access to a video-based educational program about clinical trials, located outside of this guide.

The next section in this guide is Latest Research, and it explains areas of scientific research currently going on for mastocytosis. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Mastocytosis - Latest Research

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2015

ON THIS PAGE: You will read about the scientific research being done now to learn more about mastocytosis and how to treat it. To see other pages, use the menu on the side of your screen.

Doctors are working to learn more about mastocytosis, how to best treat it, and how to provide the best care to people diagnosed with this disease. The following areas of research may include new options for patients through clinical trials. Always talk with your doctor about the diagnostic and treatment options best for you.

  • Targeted therapy. As explained in the Treatment Options section, some mast cells have a mutation of the c-kit tyrosine kinase receptor, which causes them to grow. Early results from clinical trials show that the drug imatinib and other tyrosine kinase inhibitors, such as dasatinib and nilotinib, may help treat mastocytosis. In addition, a new drug called midostaurin is also being studied for mastocytosis.

  • Palliative care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current treatments for mastocytosis in order to improve patients’ comfort and quality of life.

Looking for More About the Latest Research?

If you would like additional information about the latest areas of research regarding mastocytosis, explore these related items that take you outside of this guide:

  • To find clinical trials specific to your diagnosis, talk with your doctor or search online clinical trial databases now. Please note this link takes you outside of this guide.

  • Visit the website of the Conquer Cancer Foundation to find out how to help support clinical research. Please note this link takes you to a separate ASCO website. 

The next section in this guide is Coping with Side Effects, and it offers some guidance in how to cope with the physical, emotional, and social changes that mastocytosis and its treatment can bring. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Mastocytosis - Coping with Side Effects

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2015

ON THIS PAGE: You will find out more about steps to take to help cope with physical, social, and emotional side effects. This page includes several links outside of this guide to other sections of this website. To see other pages, use the menu on the side of your screen.

Fear of treatment side effects is common after a diagnosis of mastocytosis, but it may help to know that preventing and controlling side effects is a major focus of your health care team. This is called palliative care, and it is an important part of the overall treatment plan, regardless of the stage of disease.

There are possible side effects for every treatment, but patients don’t experience the same side effects when given the same treatments for many reasons. That can make it hard to predict exactly how you will feel during treatment.

Common side effects from each treatment option for mastocytosis are described in detail within the Treatment Options section. Learn more about the most common side effects of mastocytosis and different treatments, along with ways to prevent or control them. Side effects depend on a variety of factors, including the length and dosage of treatment(s) and your overall health.

Talking with your health care team about side effects

Before treatment begins, talk with your doctor about possible side effects of each type of treatment you will be receiving. Ask which side effects are most likely to happen, when they are likely to occur, and what can be done to prevent or relieve them.

And, ask about the level of caregiving you may need during treatment and recovery, as family members and friends often play an important role in the care of a person with mastocytosis. Learn more about caregiving.

In addition to physical side effects, there may be emotional and social effects as well. Patients and their families are encouraged to share their feelings with a member of their health care team who can help with coping strategies, including concerns about managing the cost of your medical care

During and after treatment, be sure to tell the health care team about the side effects you experience, even if you feel they are not serious. Sometimes, side effects can last beyond the treatment period, called a long-term side effect. A side effect that occurs months or years after treatment is called a late effect. Treatment of both types of effects is an important part of follow-up care. Learn more by reading Follow-Up Care section of this guide or talking with your doctor.

The next section in this guide is Follow-Up Care, and it explains the importance of check-ups after treatment is finished. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Mastocytosis - Follow-Up Care

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2015

ON THIS PAGE: You will read about your medical care after treatment is completed, and why this follow-up care is important. To see other pages, use the menu on the side of your screen.

Care for people diagnosed with mastocytosis doesn’t end when active treatment has finished. Your health care team will continue to manage any side effects, monitor your overall health, and check to make sure a mastocytosis-related cancer has not returned. This is called follow-up care.

This plan may include regular physical examinations and/or medical tests to monitor your recovery for the coming months and years. Follow-up visits will include blood tests and possibly scans or other imaging studies. Normally, follow-up visits are most frequent in the first three years after treatment, but patients are encouraged to have lifelong, follow-up care. Learn more about the importance of follow-up care.

Managing long-term and late side effects

Most people expect to experience side effects when receiving treatment. However, it is often surprising that some side effects may linger beyond the treatment period. These are called long-term side effects. In addition, other side effects called late effects may develop months or even years afterwards. Long-term and late effects can include both physical and emotional changes.

People who received ultraviolet (UV) rays for cutaneous mastocytosis should be aware of an increased risk of skin cancer in the future. Follow-up physical examinations and skin evaluations are important in the early diagnosis and prevention of this condition.

In addition, chemotherapy may cause several long-term side effects:

  • People who have received certain drugs may develop lung damage.

  • Heart damage in the form of a weakened heart muscle may occur in people who have received a higher dose of doxorubicin (Adriamycin) or radiation therapy to the chest.

  • Infertility (inability to have children) or early menopause can occur in people who have received high-dose cyclophosphamide (Cytoxan, Neosar) or other chemotherapy. Learn more about fertility concerns and preservation for men and women.

  • Secondary cancers like leukemia are more common in people who received chemotherapy. Secondary cancers are cancers that develop as a result of the treatment for another type of cancer.

Talk with your doctor about your risk of developing such side effects based on the type of mastocytosis, your individual treatment plan, and your overall health. If you had a treatment known to cause specific late effects, you may also have certain physical examinations, scans, or blood tests to help find and manage them.

Watching for recurrence

If you were treated for a mastocytosis-related cancer, one goal of follow-up care is to check for a recurrence. Cancer recurs because small areas of cancer cells may remain undetected in the body. Over time, these cells may increase in number until they show up on test results or cause signs or symptoms. During follow-up care, a doctor familiar with your medical history can give you personalized information about your risk of recurrence. Your doctor will also ask specific questions about your health. Some people may have blood tests or imaging tests as part of regular follow-up care, but testing recommendations depend on several factors including the type and stage of cancer originally diagnosed and the types of treatment given.

Keeping personal health records

You and your doctor should work together to develop a personalized follow-up care plan. Be sure to ask about any concerns you have about your future physical or emotional health. ASCO offers forms to help create a treatment summary to keep track of the treatment you received and develop a follow-up care plan once treatment is completed.

This is also a good time to decide who will lead your follow-up care. Some people continue to see a specialist, while others transition back to the general care of their family doctor or another health care professional. This decision depends on several factors, including the type of mastocytosis, side effects, health insurance rules, and your personal preferences.

If a doctor who was not directly involved in your cancer care will lead your follow-up care, be sure to share your cancer treatment summary and follow-up care plan forms with him or her, as well as all future health care providers. Details about your treatment are very valuable to the health care professionals who will care for you throughout your lifetime.

Making healthy lifestyle choices

People recovering from mastocytosis are encouraged to follow established guidelines for good health, such as not smoking, limiting alcohol, eating well, maintaining a healthy weight, managing stress, and having recommended cancer screening tests. Talk with your doctor to develop a plan that is best for your needs. Moderate physical activity can help rebuild your strength and energy level. Your doctor can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level. Learn more about making healthy lifestyle choices.

The next section offers Questions to Ask the Doctor to help start conversations with your health care team. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Mastocytosis - Questions to Ask the Doctor

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2015

ON THIS PAGE: You will find some questions to ask your doctor or other members of your health care team, to help you better understand your diagnosis, treatment plan, and overall care. To see other pages, use the menu on the side of your screen.

Talking often with the doctor is important to make informed decisions about your health care. These suggested questions are a starting point to help you learn more about your care and treatment. You are also encouraged to ask additional questions that are important to you. You may want to print this list and bring it to your next appointment, or download Cancer.Net’s free mobile app for an e-list and other interactive tools to manage your care.

Questions to ask after getting a diagnosis

  • What type of mastocytosis do I have?

  • Is the mastocytosis cancerous or benign? What does this mean?

  • Can you explain my pathology report (laboratory test results) to me?

  • Should my mast cells be tested for the c-kit mutation?

  • Are there any other skin lesions that should be biopsied?

Questions to ask about choosing a treatment and managing side effects

  • How can we manage the symptoms of mastocytosis?

  • Are there any medications or activities I should avoid?

  • What are my treatment options?

  • What clinical trials are open to me? Where are they located, and how do I find out more about them?

  • What treatment plan do you recommend? Why?

  • What is the goal of each treatment?

  • What are the possible side effects of each treatment, both in the short term and the long term?

  • Who will be part of my health care team, and what does each member do?

  • Who will be coordinating my overall treatment?

  • How will this treatment affect my daily life? Will I be able to work, exercise, and perform my usual activities?  

  • Could this treatment affect my sex life? If so, how and for how long?

  • Could this treatment affect my ability to become pregnant or have children? If so, should I talk with a fertility specialist before treatment begins?

  • If I’m worried about managing the costs related to my medical care, who can help me with these concerns?

  • What support services are available to me? To my family?

  • Whom should I call for questions or problems?

  • Is there anything else I should be asking?

Questions to ask about having surgery

  • Do I need to have surgery?

  • What type of surgery do you recommend?

  • How long will the operation take?

  • How long will I be in the hospital?

  • Can you describe what my recovery from surgery will be like? How long will it take for me to heal?

  • What are the possible long-term effects of having this surgery?

Questions to ask about having targeted therapy or chemotherapy

  • What type of treatment is recommended?

  • What is the goal of this treatment?

  • How will this treatment be given? Through an IV or orally?

  • How long will I need to continue this treatment?

  • What side effects can I expect during treatment?

  • What are the possible long-term effects of having this treatment?

  • What can be done to relieve these side effects?

Questions to ask about planning follow-up care

  • What is the risk that the mastocytosis will come back? Are there signs and symptoms I should watch for?

  • What long-term side effects or late effects are possible based on the treatment I received?

  • What follow-up tests will I need, and how often will I need them?

  • How do I get a treatment summary and follow-up care plan to keep in my personal records?

  • Who will be coordinating my follow-up care?

  • What support services are available to me after treatment? To my family?

The next section in this guide is Additional Resources, and it offers some more resources on this website beyond this guide that may be helpful to you. Or, use the menu on the side of your screen to choose another section to continue reading this guide.  

Mastocytosis - Additional Resources

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2015

ON THIS PAGE: You will find some helpful links to other areas of Cancer.Net that provide information about medical care and treatment. This is the final page of Cancer.Net’s Guide to Mastocytosis. To go back and review other pages, use the menu on the side of your screen.

Cancer.Net includes many other sections about the medical and emotional aspects of cancer and cancer-related conditions, both for the person diagnosed and their family members and friends. This website is meant to be a resource for you and your loved ones from the time of diagnosis, through treatment, and beyond.

Beyond this guide, here are a few links to help you explore other parts of Cancer.Net:

This is the end of Cancer.Net’s Guide to Mastocytosis. Use the menu on the side of your screen to select another section to continue reading this guide.