Three Types of Systemic Mastocytosis (SM)

A rare condition that can be hard to diagnose

Advanced systemic mastocytosis (SM)* is a rare condition that can be hard to diagnose. Patients are often referred to different specialists, such as allergists and dermatologists, without getting the correct diagnosis and treatment. This can be a long and frustrating experience for patients and their families. But once a diagnosis is reached, the disease may be properly treated.

*Aggressive systemic mastocytosis (ASM), systemic mastocytosis with associated hematological neoplasm (SM-AHN), or mast cell leukemia (MCL) are 3 types of SM and are collectively referred to as advanced SM.

Talking to your doctor

If you have been experiencing the signs and symptoms listed here and they have not gone away, it is important to talk to your doctor.

talking to your doctor about advanced SM

Communicate regularly with your health care provider so you can make informed decisions about your treatment. Below are some questions that may help you start a conversation with your doctor about treatment. Be sure to bring up any additional questions that may help you better understand how to get the most out of your treatment, as all of your questions are important.

It is always a good idea to bring a loved one or caregiver with you when you see your doctor. You will probably get a lot of information at once. Having someone there with you to take notes and ask questions can help you stay organized and help you stay positive.

  • What symptoms or side effects should I tell you about right away?
  • How will I know if my treatment with RYDAPT® (midostaurin) capsules is working?
  • What if I forget to take RYDAPT?
  • Do I need to change my diet during treatment with RYDAPT?
  • Is monitoring required while I’m taking RYDAPT?
  • What if I stop taking RYDAPT?
  • What if I take too much RYDAPT?
  • How long will I need to stay on RYDAPT?
  • What should I know about taking RYDAPT while sexually active?
  • What if I’m pregnant or lactating, or plan on becoming pregnant?

What tests are there for advanced SM?

Your health care team will need to do several tests to diagnose advanced SM. These may include blood tests, a skin biopsy, and testing your bone marrow.


Your health care team may also test for a genetic mutation called KIT D816V. About 9 out of every 10 patients with advanced SM have this mutation. Finding out if you have this mutation can help your health care team give you an accurate diagnosis and come up with an appropriate treatment plan.