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What Is Advanced Systemic Mastocytosis? Everything To Know

Advanced systemic mastocytosis is a progressive disorder that causes the atypical buildup of mast cells in different organs in the body. Treatment aims to slow the progression of the condition and relieve symptoms.

Some symptoms of advanced systemic mastocytosis include headache, diarrhea, hypotension, and more. Doctors may order various tests to diagnose the condition and advise on a suitable treatment plan.

Read on to learn more about advanced systemic mastocytosis, including the symptoms, causes, and more.

Advanced systemic mastocytosis is an advanced form of the condition that may cause different signs and symptoms, including:

The American Academy of Allergy Asthma and Immunology (AAAAI) notes that people may experience multiple symptoms as the condition progresses, affecting different organs. Without early diagnosis and treatment, advanced systemic mastocytosis may lead to tissue damage and organ failure, affecting life expectancy.

Genetic mutation of the KIT genes plays a role in the development of advanced systemic mastocytosis, leading to the atypical buildup of mast cells in one or more tissues.

The KIT genes control chemical signaling pathways that instruct the growth of different cells in the body, including mast cells. Mast cells prevent foreign body invasion through inflammatory responses.

Certain factors may activate the mast cells. Possible triggers include:

  • friction
  • minor injury
  • surgery
  • temperature changes
  • insect stings
  • some vaccines
  • anxiety or stress
  • certain medications, such as opioids, aspirin, and nonsteroidal anti-inflammatory drugs
  • A doctor may perform several examinations and tests to help diagnose advanced systemic mastocytosis. These include the following:

  • Blood test: Complete blood count and elevated tryptase levels can help differentiate the variants of advanced systemic mastocytosis. Tryptase is an enzyme that primarily comes from mast cells.
  • Bone marrow biopsy: Using a long needle, the doctor will remove a sample of bone marrow tissue for further testing. A person will receive a local anesthetic before this procedure.
  • Imaging tests: Imaging tests, such as ultrasounds, can help the doctor view internal organs and tissues clearly. Additionally, dual-energy X-ray absorptiometry scans can help assess bone density.
  • A person's doctor can advise on what tests they order and what they involve.

    Learn about bone marrow biopsies.

    Treatment for advanced systemic mastocytosis aims to slow the progression of the condition and relieve symptoms. What symptoms a person experiences will help determine their treatment.

    Examples of possible treatments for symptoms include:

  • antihistamines for symptoms such as abdominal pain and diarrhea
  • oral cromolyn sodium for gastrointestinal symptoms
  • pamidronate and low dose interferon-alfa for osteoporosis
  • Medications a doctor may recommend for the aggressive form of systemic mastocytosis include:

  • cladribine
  • midostaurin
  • interferon-alpha
  • tyrosine kinase inhibitors
  • A person's doctor can advise on what treatment they recommend and can help them create a treatment plan to manage their symptoms.

    Possible complications of systemic mastocytosis include:

  • anemia
  • coagulopathy
  • polycythemia vera, which is an increase of red blood cells in the body
  • Castleman disease, a condition that involves noncancerous enlargement of lymph node tissue
  • monoclonal gammopathy
  • A person's doctor can advise on ways they can manage the condition and reduce the risk of complications.

    Here are answers to common questions about systemic mastocytosis.

    What is the prognosis for advanced systemic mastocytosis?

    Without treatment, people with advanced systemic mastocytosis may have a negative outlook. A 2021 study examining the response criteria of advanced systemic mastocytosis found that administering KIT inhibitors such as avapritinib and midostaurin improved this outlook.

    It is important to note that each individual's outlook will differ. A person's doctor can provide more information about their outlook.

    What is the life expectancy of someone with aggressive systemic mastocytosis?

    People with aggressive systemic mastocytosis may have a lower life expectancy than those without the condition or individuals with a less aggressive form of it. However, as with the outlook in general, life expectancy can vary from person to person.

    Advanced systemic mastocytosis is a progressive disorder that causes the atypical buildup of mast cells in different organs in the body.

    People with advanced systemic mastocytosis may have a negative outlook and lower life expectancy without treatment. However, treatment may offer benefits to those with the condition. Treatment for advanced systemic mastocytosis can help slow down the progression of the condition and help with managing symptoms.

    It is best to contact a doctor as soon as there are concerns about advanced systemic mastocytosis. They can order tests to confirm the diagnosis and advise on suitable treatments.


    How Long Does It Take For STD Symptoms To Show?

    STDs, or STIs, usually pass from person to person through sexual contact. In most cases, a person can get an STI test within a few weeks of exposure.

    Each STI has its own incubation period, which is how long it takes for symptoms to appear. In some cases, it can take months for an STI to show up on tests. In other cases, it may only take days.

    This article explores the incubation periods of different STIs, how soon people can get tested, and the importance of testing.

    The incubation period is how long it takes for symptoms to appear after exposure. The window period is how long it takes to get a positive test result for the infection after exposure. These periods are often similar.

    Some general symptoms that indicate a person might have an STI include:

  • genital itching or burning
  • pain during intercourse or urination
  • a new or unusual discharge
  • bumps or growths on or around the genitals
  • a foul smell coming from the genitals or after sex
  • However, some STIs do not cause symptoms for many years, even though a person can still get a positive test result. This is why it is important to rely on testing, not just symptoms.

    In most cases, a person can get an STI test within a few weeks of exposure. If a person has a curable STI, such as chlamydia or gonorrhea, they may need a retest after treatment.

    People at high risk of certain STIs should ask for a retest, even after a negative result. For example, the Centers for Disease Control and Prevention (CDC) recommend annual HIV testing for people at risk, such as those whose partners have HIV or people who share needles.

    The testing window for common STIs is as follows:

    HIV

    A nucleic acid test analyzes a blood sample for HIV. It can indicate a positive result 10–33 days after exposure. The antigen/antibody test, also a blood test, looks for HIV antibodies. It also looks for an antigen that the body produces before antibodies appear. It can get results 18–45 days after exposure.

    The antibody test uses a blood or saliva sample to look for HIV antibodies. It takes the longest to get a reliable result, at 23–90 days after exposure. A person can be confident they do not have HIV if they get a negative test during the window period and have no subsequent contact with someone who could have the virus.

    Chlamydia

    A doctor can test for chlamydia by swabbing the vagina, cervix, rectum, or throat, or by taking a urine sample. If symptoms appear, they usually present within 7–21 days of exposure. A test can normally detect chlamydia within 1–2 weeks of exposure.

    Gonorrhea

    A doctor can test for gonorrhea with a urine sample. In some cases, they may also swab the urethra, anus, throat, or cervix to get a more reliable result.

    Most tests can detect the infection within 5 days to 2 weeks of exposure. If a test is negative shortly after exposure, a doctor may recommend retesting 2 weeks later, particularly if a person has symptoms.

    Gonorrhea symptoms usually appear from 1 day to 2 weeks after exposure.

    Herpes

    Herpes symptoms usually appear quickly. On average, they present 4 days after exposure, and the typical range is 2–12 days. In some cases, however, symptoms can be so mild that a person does not notice them.

    A blood test can confirm a herpes diagnosis. The test may be positive within a month, and by 4 months, blood testing finds most cases.

    HPV

    While it is possible for males to pass human papillomavirus (HPV) on to a partner, the CDC have not approved a male test. Instead, doctors may test for symptoms of the relatively rare cancers that HPV can cause, including penile cancer.

    In females, HPV rarely causes symptoms. If there are indications, they could appear months or years later. The most reliable test is a Pap smear, which involves swabbing the cervix. This can detect HPV 3 weeks to a few months after exposure.

    Hepatitis

    In some cases, hepatitis B and C may not cause any obvious symptoms for years. If they do appear, hepatitis B usually produces signs within 6 weeks to 6 months. Hepatitis C symptoms may appear as early as 2–6 weeks, but can sometimes take as long as 6 months.

    A blood test can look for both types of hepatitis. The hepatitis B testing window is 3–6 weeks, while the hepatitis C testing window is 2–6 months. Early testing at 2 months may miss some cases, so a doctor may recommend retesting at 6 months.

    Trichomoniasis

    A doctor can test for trichomoniasis with a swab of the rectum, penis, or vagina. Many people do not have symptoms, but some may notice a discharge or burning sensation within 5–28 days of exposure. It is possible to get a positive test within a week of exposure, though some people need to wait up to a month.

    Syphilis

    Syphilis usually begins with a sore on the genitals called a chancre. Blood tests can detect the bacteria within 1–2 weeks after the chancre appears. Chancres are typically painless and usually develop within 3 weeks of exposure, so the total testing window is about 4 weeks.

    Because the development of syphilis varies from person to person, doctors often recommend retesting about 3 months after exposure.

    Some STIs live in the body for many years without triggering any symptoms. Doctors may refer to them as dormant, meaning a person can never diagnose themselves based on symptoms alone.

    This also means if a person is untested, they can unknowingly pass a dormant STI on to a sexual partner.

    Examples of STI that can lay dormant include HIV, herpes, hepatitis C, chlamydia, syphilis, and HPV

    The CDC recommend that all sexually-active adults with new or multiple partners seek testing for most STIs at least once per year.

    STI testing, even for incurable infections, can save lives. It also slows the spread of STIs. Some benefits of testing are below:

  • Some STIs are easier to treat if a doctor catches them early.
  • Early STI testing can prevent a person from spreading an infection to their partners.
  • A person can have an STI without knowing it.
  • Some untreated STIs can cause serious health issues, such as cancer and pelvic inflammatory disease.
  • The right STI testing depends on many factors, including a person's medical history, sexual history, risk of exposure, and prior history of STI tests.

    It is important for people to regularly test for STIs, particularly if they have sex with multiple partners.

    Early detection can make treatment easier and possibly prevent serious health issues. To reduce the risk of STIs, people should also practice safer sex techniques, such as using a condom.


    Gonorrhea Directory

    Also called the "clap" or "drip," gonorrhea is a sexually transmitted disease (STD). It can also be transmitted during pregnancy from a mother to her baby. Gonorrhea is a common STD, especially among teens. Symptoms include a green or yellow discharge, painful urination, abdominal pain, and more. Follow the links below to find WebMD's comprehensive coverage about how gonorrhea is contracted, how to treat it, and much more.

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