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• #1 Systemic Mastocytosis

  https://www.aaaai.org/conditions-treatments/related-conditions/systemic-mastocytosis

  Most adults with systemic mastocytosis have infiltration of one or more internal organs with abnormal mast cells and can present with or without cutaneous mastocytosis. […] Mastocytosis may be suspected when there are persistent symptoms of mast cell mediator release, especially in the absence of known triggers of mast cell mediator release, such as allergy or certain medications. Patients may experience one or more of the symptom groups listed. Symptoms of Mastocytosis include: Anaphylaxis Itching, flushing, hives, swelling Wheezing or shortness of breath Sinus congestion and pressure Throat swelling Palpitations, changes in blood pressure, dizziness, fainting Nausea, vomiting, abdominal pain, diarrhea Uterus cramps/bleeding Bone or muscle pain, osteopenia, osteoporosis Headache, brain fog, anxiety, short memory span, depression

• #2 Systemic mastocytosis: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/systemic-mastocytosis/

  Systemic mastocytosis is a blood disorder that can affect many different body systems. Individuals with the condition can develop signs and symptoms at any age, but it usually appears after adolescence. Signs and symptoms of systemic mastocytosis often include extreme tiredness (fatigue), skin redness and warmth (flushing), nausea, abdominal pain, bloating, diarrhea, the backflow of stomach acids into the esophagus (gastroesophageal reflux), nasal congestion, shortness of breath, low blood pressure (hypotension), lightheadedness, and headache. Some affected individuals have attention or memory problems, anxiety, or depression. Many individuals with systemic mastocytosis develop a skin condition called urticaria pigmentosa, which is characterized by raised patches of brownish skin that sting or itch with contact or changes in temperature. Nearly half of individuals with systemic mastocytosis will experience severe allergic reactions (anaphylaxis).

• #3 Systemic mastocytosis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/systemic-mastocytosis/symptoms-causes/syc-20352859

  Signs and symptoms of systemic mastocytosis depend on the part of the body affected by excessive mast cells. Too many mast cells can build up in the skin, liver, spleen, bone marrow or intestines. Less commonly, other organs such as the brain, heart or lungs also may be affected. […] Signs and symptoms of systemic mastocytosis may include: Flushing, itching or hives, Abdominal pain, diarrhea, nausea or vomiting, Anemia or bleeding disorders, Bone and muscle pain, Enlarged liver, spleen or lymph nodes, Depression, mood changes or problems concentrating. […] The mast cells are triggered to produce substances that cause inflammation and symptoms. People have different triggers, but the most common ones include: Alcohol, Skin irritation, Spicy foods, Exercise, Insect stings, Certain medications.

• #4 Systemic mastocytosis – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/systemic-mastocytosis/

  Signs and symptoms of systemic mastocytosis depend on the part of the body affected by excessive mast cells. Too many mast cells can build up in the skin, liver, spleen, bone marrow or intestines. Less commonly, other organs such as the brain, heart or lungs also may be affected. […] Signs and symptoms of systemic mastocytosis may include: Flushing, itching or hives, Abdominal pain, diarrhea, nausea or vomiting, Anemia or bleeding disorders, Bone and muscle pain, Enlarged liver, spleen or lymph nodes, Depression, mood changes or problems concentrating. […] The mast cells are triggered to produce substances that cause inflammation and symptoms. People have different triggers, but the most common ones include: Alcohol, Skin irritation, Spicy foods, Exercise, Insect stings, Certain medications.

• #5 About Systemic Mastocytosis | Blueprint Medicines

  https://itssmthing.com/about-sm.php

  Some symptoms of SM may seem like common intolerances, or new allergies (for example, developing sudden additional allergies to milk or shellfish, etc.). People with SM sometimes manage their condition by following a strict diet and routinely avoiding triggers at work or in their social lives. […] People with SM may experience different symptoms that may be unexplained, unpredictable, recurring, or severe. Here are some examples: […] Hives, rashes that look like spots, flushing that lasts more than a few minutes. […] Diarrhea, nausea, heartburn, abdominal cramps, sensitivities to food. […] Severe or whole-body allergic reactions, including anaphylaxis or bone pain. […] SM is a chronic (life-long) condition with symptoms that vary greatly from person to person. Advanced forms of SM (AdvSM) can be associated with progressive (worsening) organ damage. There is approximately a 5% chance ISM may progress to SSM or AdvSM and approximately a 9% chance SSM may progress to AdvSM.

• #6 Mastocytosis: Types, Symptoms, Causes, Diagnosis, Treatment and More

  https://www.health.com/mastocytosis-8415852

  This type of mastocytosis can lead to a wide range of symptoms, such as fatigue, skin flushing (redness and warmth), nausea, headache, abdominal pain, gastroesophageal reflux, nasal congestion, hypotension (low blood pressure), bone pain, and shortness of breath. […] Symptoms of mastocytosis may initially be mild but can worsen in severity as mast cells build up in your body. Though symptoms can vary depending on the type of mastocytosis a person has, each type shares common symptoms, including: Skin flushing (redness and warmth), especially in the face, itchy and raised hives on the skin, abdominal pain, diarrhea, shortness of breath, syncope (fainting), wheezing, irritability, memory problems or difficulty concentrating, anaphylaxis (severe allergic reactions). […] Systemic mastocytosis may affect multiple organ systems, including the skin, and can cause symptoms such as: fatigue, skin flushing, nausea, bloating, abdominal pain, diarrhea, acid reflux, hypotension (low blood pressure), headache, lightheadedness, heart palpitations, bone pain, anxiety, depression, memory problems, trouble focusing, reddish-brown patches on the skin that may sting or itch, severe allergic reactions.

• #7 Symptoms and Triggers of Mast Cell Activation – TMS – The Mast Cell Disease Society, IncAccessibilityIncrease TextDecrease TextGrayscaleHigh ContrastNegative ContrastLight BackgroundLinks UnderlineReadable FontReset

  https://tmsforacure.org/signs-symptoms-triggers/symptoms-and-triggers-of-mast-cell-activation/

  Mast cells can be activated to release mediators by multiple triggers. […] The myriad symptoms patients with mast cell diseases experience during mast cell activation can wreak havoc on patients on a daily basis, and multiple organ systems, including pulmonary, cardiovascular, dermatologic, gastrointestinal, musculoskeletal, and neurologic can be involved. Symptoms may include, but are not limited to: flushing of the face, neck, and chest; headache; tachycardia and chest pain; abdominal pain, bloating, gastroesophageal reflux disease (GERD), diarrhea, vomiting; uterine cramps or bleeding; rashes, including maculopapular cutaneous mastocytosis (MPCM)/urticaria pigmentosa (UP), telangiectatic lesions; bone/muscle pain, osteosclerosis, osteopenia, osteoporosis; itching, +/- rash; blood pressure instability; brain fog, cognitive dysfunction; anxiety/depression; lightheadedness, syncope; and anaphylaxis. […] It should be noted that the manifestation of anaphylaxis or similar symptoms among infants and preschoolers may be more difficult to identify. […] Anaphylaxis is an acute life-threatening systemic reaction that results from the sudden, rapid, systemic release of mediators.

• #8 Azthena logo with the word Azthena

  https://www.news-medical.net/health/What-is-Systemic-Mastocytosis.aspx

  Symptoms can be either due to the release of MC mediators or organ damage caused by MC invasion. Fatigue, nausea, skin redness and warmth, abdominal pain, bloating, and diarrhea are common signs and symptoms of systemic mastocytosis. […] Other typical symptoms include nasal congestion, shortness of breath, hypotension, lightheadedness, and headache. Some people may suffer from attention or memory issues, as well as anxiety or depression. […] Many individuals with systemic mastocytosis develop urticaria pigmentosa, characterized by raised areas of brownish skin that sting or itch when touched or when the temperature changes. […] Nearly 50% of people with systemic mastocytosis will have severe allergic responses (anaphylaxis). Gastrointestinal symptoms, such as stomach acid backflow into the esophagus, are prevalent in 60-80% of SM patients and are one of the leading causes of morbidity.

• #9 Gastrointestinal manifestations of systemic mastocytosis

  https://www.wjgnet.com/1007-9327/full/v14/i45/7005.htm

  Systemic mastocytosis (SM) is a rare disease with abnormal proliferation and infiltration of mast cells in the skin, bone marrow, and viscera including the mucosal surfaces of the digestive tract. Gastrointestinal (GI) symptoms occur in 14%-85% of patients with systemic mastocytosis. The GI symptoms may be as frequent as the better known pruritus, urticaria pigmentosa, and flushing. In fact most recent studies show that the GI symptoms are especially important clinically due to the severity and chronicity of the effects that they produce. GI symptoms may include abdominal pain, diarrhea, nausea, vomiting, and bloating. […] Different studies report a wide array of GI involvement with a range from 14%-85% and more recent studies suggest that it comes as a close second commonest symptom next only to pruritus.

• #10 Gastrointestinal manifestations of systemic mastocytosis

  https://www.wjgnet.com/1007-9327/full/v14/i45/7005.htm

  It must be stressed that while most patients have pruritus or urtication, these symptoms generally cause less significant discomfort for patients than the GI symptoms which are more distressing chronic complaints. […] Common GI complaints in SM include abdominal pain, diarrhea, nausea, and vomiting. It appears that the frequency of various GI symptoms is less variable than their prevalence. These symptoms are thought to be secondary to mast cell mediators on the GI tract. The most common symptom is abdominal pain (mean 51%) followed by diarrhea (mean 43%) and nausea or vomiting (mean 28%). […] Diarrhea is thought to occur due to gastric acid hypersecretion, malabsorption caused by mucosal injury and edema, and altered bowel motility. […] A common symptom in SM is facial flushing. […] Other symptoms may also occur as a result of GI bleeding and peptic ulcer disease. It is estimated that GI bleeding occurs in 11% of SM.

• #11 Systemic Mastocytosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK544345/

  Systemic mastocytosis is an aggressive disorder characterized by the release of numerous vasoactive cell mediators due to excessive activity of mast cells, which results in a wide variety of symptoms. Symptoms include anaphylaxis, flushing, nonspecific GI as well as neuropsychiatric complaints. […] The most frequent symptoms associated with the disease include pruritis, flushing, itching, diarrhea, and anaphylaxis. […] Acute release of mast cell mediators can result in episodes of vasodilation, hypotension, flushing, pruritus, syncope, abdominal pain, nausea, vomiting, diarrhea, fatigue, and headache. […] Anaphylaxis is observable in both cutaneous mastocytosis and systemic mastocytosis. Symptoms typically include flushing, syncope, gastrointestinal symptoms, and vascular collapse. […] Gastrointestinal dysfunction caused by the release of mast cell mediators is an observation in systemic mastocytosis.

• #12 What is Mastocytosis? Types, Symptoms, and Treatment – Allergy & Asthma Network

  https://allergyasthmanetwork.org/mast-cell-diseases/mastocytosis/

  Mastocytosis is a rare disorder and a type of mast cell disease. It is a condition in which there are too many mast cells in the body. […] Systemic mastocytosis involves organs and tissues across the whole body. It is more common in adults than children. The symptoms may include (but are not limited to) the skin, digestive tract, liver, spleen and bone marrow. The condition is most common in adults, but some children also develop the condition. […] Systemic mastocytosis can present with more symptoms. Symptoms may involve the skin, bone marrow, liver, spleen, or digestive tract. People with systemic mastocytosis may develop bone pain and fatigue. They may also be at risk for a severe or life-threatening allergic reactions, or anaphylaxis. […] Symptoms reflect the body system involved. It may affect a small area of your body (local). It could affect several body systems (systemic). Symptoms may include: Skin: rash or lesions, swelling, flushing; Breathing: shortness of breath; Stomach: abdominal pain, nausea, vomiting or diarrhea, GERD; Throat: swelling; Brain: headache, brain fog, memory issues, depression, mood changes; Heart: palpitations, blood pressure changes, dizziness, fainting; Bones: bone pain, bone density loss, bone fracture (in severe cases); Blood: anemia, bleeding issues; Organs: enlarged spleen, liver, or lymph nodes; Severe allergic reactions (anaphylaxis).

• #13 Systemic Mastocytosis Symptoms | Blueprint Medicines

  https://itssmthing.com/symptoms.php

  There may be a variety of unexplained, recurring symptoms of systemic mastocytosis (SM) that can span across multiple organ systems. […] SM is associated with a range of symptoms throughout the body. Symptoms vary in nature since they can be severe, unpredictable, unexplained, and recurring. It’s important to remember that different people may experience different symptoms. […] The following are some of the common symptoms of SM: […] Abdominal pain or cramping, recurring diarrhea, heartburn or acid reflux, nausea, vomiting. […] Low blood pressure (hypotension), heart flutters (palpitations), rapid heart rate (tachycardia), chest pain, dizziness or light-headedness, fainting from anaphylaxis. […] Nasal congestion, shortness of breath, wheezing, throat itching, or swelling. […] Bone pain, muscle pain, bone loss, weak and brittle bones that may break or fracture easily.

• #14 Systemic Mastocytosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK544345/

  Neuropsychiatric manifestations, including depression, mood changes, lack of concentration, short memory span, increased somnolence, irritability, as well as emotional instability, are commonly witnessed in adult patients with mastocytosis. […] Diffuse musculoskeletal pain of the long bones and a pain syndrome resembling fibromyalgia may appear in patients with systemic mastocytosis. […] A subset of patients with systemic mastocytosis appears to be at increased risk of developing osteopenia and osteoporosis. […] The most common hematologic abnormality is mild-to-moderate anemia, which occurs in up to 50 percent of patients. […] Peptic ulcer disease, malabsorption, steatorrhea, and liver enlargement commonly present when mast cell infiltration occurs in the intestine or the liver in aggressive systemic mastocytosis (ASM).

• #15 Systemic Mastocytosis – MD Searchlight

  https://mdsearchlight.com/health/systemic-mastocytosis/

  Gastrointestinal complaints: Problems related to the digestive system due to the release of mast cell chemicals are common in systemic mastocytosis. Symptoms can include abdominal pain, diarrhea, nausea, vomiting, peptic ulcer disease, and gastrointestinal bleeding. […] Neuropsychiatric symptoms: Emotional and cognitive effects are commonly seen in adult patients. They may suffer from depression, mood changes, decreased focus, poor short-term memory, increased sleepiness, irritability, and emotional instability. […] Musculoskeletal symptoms: Diffuse pain in large bones and a pain type similar to fibromyalgia may occur. Also, decreased bone density is seen in some patients, putting them at risk of developing osteopenia and osteoporosis. […] Gastrointestinal and liver abnormalities: Peptic ulcer disease, malabsorption, fatty stools, and liver enlargement are often observed when mast cells infiltrate the intestine or liver, particularly in a severe form of the disease.

• #16

  https://111.wales.nhs.uk/mastocytosis/

  People with mastocytosis have an increased risk of developing a severe and life-threatening allergic reaction. This is known as anaphylaxis. […] If you have systemic mastocytosis or extensive cutaneous mastocytosis, your risk of a severe allergic reaction (anaphylaxis) is increased. […] The most common symptoms experienced during an episode are: hot flushing, a forceful or rapid heartbeat (heart palpitations), feeling lighthead. […] In more severe cases of mastocytosis, the following symptoms may occur: weight loss, swelling of the lymph nodes, swelling of the liver which can cause jaundice and make you feel lethargic, swelling of the spleen which can cause tummy and shoulder pain. […] Some people with severe symptoms experience a sudden fall in blood pressure during an attack. […] Low blood pressure (hypotension) can trigger a number of associated symptoms, such as: dizziness, fainting, blurred vision, confusion, general weakness.

• #16

  https://111.wales.nhs.uk/mastocytosis/

  Mastocytosis is a rare condition caused by an excess number of mast cells gathering in the body’s tissues. […] Systemic mastocytosis, which mainly affects adults, where mast cells gather in body tissues, such as the skin, internal organs and bones. […] Some people with systemic mastocytosis may experience episodes of severe symptoms, often with specific triggers such as physical exertion or stress. Many people do not have any problems. […] During an episode you may have: skin reactions such as itching and flushing, gut symptoms such as being sick and diarrhoea, muscle and joint pain, mood changes, headaches and episodes of tiredness (fatigue). […] There are 3 subtypes of systemic mastocytosis. They are: indolent mastocytosis symptoms are usually mild to moderate and vary from person to person; indolent mastocytosis accounts for around 90% of adult systemic mastocytosis cases, aggressive mastocytosis where mast cells multiply in organs, such as the spleen, liver and digestive system; the symptoms are more wide-ranging and severe, although skin lesions are less common, systemic mastocytosis with associated blood (haematological) disease where a condition that affects the blood cells, such as chronic leukaemia, also develops.

• #17 Systemic Mastocytosis | Hôpital Erasme

  https://www.erasme.be/en/health-problems/systemic-mastocytosis

  The signs and symptoms of systemic mastocytosis can include flushing, itching. Abdominal pain, diarrhoea, nausea or vomiting. Palpitations, feeling faint. Allergic reactions ranging from urticaria to Quinckes oedema and anaphylaxis. Coughing, shortness of breath, rhinorrhea, lachrymation. Frequent urination, urinary burning. Bone and muscle pain. Depression, mood changes or problems concentrating. Anaemia or bleeding disorders. Enlarged liver, spleen or lymph nodes. […] To diagnose systemic mastocytosis a biopsy of the organ or organs affected is necessary to establish the link between the symptoms and the illness. The biopsy is to detect the presence of an accumulation of mast cells as well as the c-KIT mutation. The tryptase level is a blood marker that is very often useful for the diagnosis.

• #18 Mastocytosis: Understanding Causes, Symptoms & Treatment

  https://juniperallergy.com/mastocytosis-causes-symptoms-treatment/

  The symptoms of Mastocytosis can vary widely depending on the type and extent of the condition. Some common signs and symptoms include: […] Skin-related symptoms: Pruritus (itching), urticaria (hives), flushing (reddening of the skin), and skin lesions like brownish spots or nodules. […] Gastrointestinal symptoms: Abdominal pain, nausea, vomiting, diarrhea, and peptic ulcers. […] Cardiovascular symptoms: Hypotension (low blood pressure), tachycardia (rapid heart rate), and even anaphylaxis (a severe allergic reaction). […] Respiratory symptoms: Shortness of breath, wheezing, and chest tightness. […] Bone and joint pain: Especially prevalent in adults with systemic mastocytosis. […] Fatigue, headache, and neuropsychiatric symptoms: Cognitive impairment, mood swings, and irritability.

• #19 Systemic Mastocytosis: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/203948-overview

  Systemic mastocytosis, often termed systemic mast cell disease (SMCD), is characterized by infiltration of clonally derived mast cells in different tissues, including bone marrow, skin, the gastrointestinal (GI) tract, the liver, and the spleen. Median survival ranges from 198 months in patients with indolent systemic mastocytosis to 41 months in aggressive systemic mastocytosis and 2 months in acute mast cell leukemia. […] Manifestations of systemic mastocytosis may include the following: Anemia and coagulopathy, Abdominal pain is the most common GI symptom, followed by diarrhea, nausea, and vomiting, Symptoms and signs of gastroesophageal reflux disease (GERD), Pruritus and flushing, Anaphylactoid reaction (eg, to Hymenoptera stings, general anesthetics, intravenous contrast media, other drugs, foods), Neuropsychiatric manifestations (eg, depression, mood changes, lack of concentration, short memory span, increased somnolence, irritability, and emotional instability), Increased risk of developing osteopenia and osteoporosis.

• #20 Systemic Mastocytosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK544345/

  Central and peripheral lymphadenopathy occurs in 20 to 60 percent of patients with indolent systemic mastocytosis (ISM) and a higher proportion in those with systemic mastocytosis with an associated hematologic neoplasm (SM-AHN), ASM, and mast cell leukemia (MCL). […] Splenomegaly can present in 50 percent of patients with ISM and over 70 percent of those with SM-AHN, ASM, and MCL. […] The disease subtype is one of the strongest prognostic predictors in systemic mastocytosis. […] Certain clinical features are associated with an increased risk of death due to disease progression, independent of the category of systemic mastocytosis present.

• #21 Systemic mastocytosis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/systemic-mastocytosis/symptoms-causes/syc-20352859

  Complications of systemic mastocytosis can include: Anaphylactic reaction. This severe allergic reaction includes signs and symptoms such as rapid heartbeat, fainting, loss of consciousness and shock. If you have a severe allergic reaction, you may need an injection of epinephrine. Blood disorders. These can include anemia and poor blood clotting. Peptic ulcer disease. Chronic stomach irritation can lead to ulcers and bleeding in your digestive tract. Reduced bone density. Because systemic mastocytosis can affect your bones and bone marrow, you may be at risk of bone problems, such as osteoporosis. Organ failure. A buildup of mast cells in body organs can cause inflammation and damage to the organ.

• #22 Advanced systemic mastocytosis

  https://www.medicalnewstoday.com/articles/advanced-systemic-mastocytosis

  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. […] Advanced systemic mastocytosis is an advanced form of the condition that may cause different signs and symptoms, including: nausea, diarrhea, hypotension or low blood pressure, peptic ulcer disease, rash-like skin lesions, osteoporosis, osteopenia, lymphadenopathy, or swollen lymph nodes, hepatosplenomegaly, or an enlarged liver and spleen, dysplasia, or the atypical growth of cells, marked cytopenia, or a reduced number of blood cells, osteolysis, or bone degeneration, ascites, where fluid collects in the abdomen, malabsorption.

• #23 Diagnostic Workup for Advanced Forms of Mastocytosis – TMS – The Mast Cell Disease Society, IncAccessibilityIncrease TextDecrease TextGrayscaleHigh ContrastNegative ContrastLight BackgroundLinks UnderlineReadable FontReset

  https://tmsforacure.org/expert-information/diagnostic-workup-advanced-forms-mastocytosis/

  Mastocytosis denotes a heterogeneous group of disorders characterized by abnormal growth and accumulation of mast cells (MCs) that has a variable prognosis and course of disease. […] Most systemic mastocytosis (SM) variants diagnosed in adults show a stable clinical course over decades and do not decrease the life span. […] The presence or absence of skin lesions can be an important clinical finding. Patients with ASM or MCL may or may not have skin lesions, with less than 50% of those with MCL presenting with cutaneous involvement. […] Another observation of importance is weight loss. Together with hypoalbuminemia, it is indicative of mast cell-induced organ damage and thus is a C-finding. […] An additional typical finding in SM is symptoms caused by the release of mast cell mediators, including histamine, cytokines, and leukotrienes. Related symptoms are headache, flushing, pruritus, diarrhea, hypotension, and bone pain. Excessive mast cell mediator release may lead to anaphylaxis in patients with mastocytosis.

• #24

  https://www.nhs.uk/conditions/mastocytosis/

  People with mastocytosis have an increased risk of developing a severe and life-threatening allergic reaction. This is known as anaphylaxis. […] The increased risk of anaphylaxis is caused by the abnormally high number of mast cells and their potential to release large amounts of histamine into the blood. […] The outlook for systemic mastocytosis can vary, depending on the type you have. Indolent systemic mastocytosis should not affect life expectancy, but other types can.

• #24

  https://www.nhs.uk/conditions/mastocytosis/

  Some people with systemic mastocytosis may experience episodes of severe symptoms, often with specific triggers such as physical exertion or stress. Many people do not have any problems. […] During an episode you may have: skin reactions such as itching and flushing, gut symptoms such as diarrhoea and vomiting, muscle and joint pain, mood changes, headaches and tiredness (fatigue). […] There are 3 main subtypes of systemic mastocytosis. They are: indolent systemic mastocytosis symptoms are usually mild to moderate and vary from person to person; indolent systemic mastocytosis accounts for around 90% of adult systemic mastocytosis cases, aggressive mastocytosis where mast cells multiply in organs, such as the spleen, liver and digestive system; the symptoms are more wide-ranging and severe, although skin lesions are less common, systemic mastocytosis with associated blood (haematological) disease where a condition that affects the blood cells, such as chronic leukaemia, also develops.

• #25 Diagnosis and treatment of systemic mastocytosis in Brazil: Recommendations of a multidisciplinary expert panel | Hematology, Transfusion and Cell Therapy

  http://www.htct.com.br/en-diagnosis-treatment-systemic-mastocytosis-in-articulo-S2531137922000803

  The D816V KIT mutation allele burden was also found to correlate with disease activity, disease subtype and survival. […] The treatment of SM should be individualized and varies from a watch-and-wait period to the symptom management, supportive measures and cytoreductive therapy for MC debulking in the setting of aggressive, advanced or treatment-refractory disease. […] The symptomatic treatment aims to manage symptoms efficiently and minimize their recurrence. The avoidance of triggers and use of prophylactic medication, when risk avoidance is difficult or impossible, are necessary first measures for symptomatic control. […] Mastocytosis symptoms can be divided into skin symptoms, mast cell mediator release symptoms and symptoms caused by non-cutaneous organ infiltration. […] The anaphylaxis is a significant complication in mastocytosis patients. Although the risk of anaphylaxis is less than 10% in children with CM, it is estimated to be 50% in adults with ISM. […] The ASM has a worse prognosis than ISM, presenting with C findings, organ dysfunctions and, less frequently, cutaneous disease.

• #26 Systemic Mastocytosis: Symptoms, Subtypes, Treatment

  https://www.verywellhealth.com/systemic-mastocytosis-8669227

  Systemic mastocytosis encompasses a spectrum of symptoms that can vary widely in severity and presentation across different people. These symptoms predominantly arise from the excessive activation and release of mast cells, affecting various organ systems in the body. […] Emergency symptoms of systemic mastocytosis can be severe and require immediate medical attention. These include: Anaphylaxis, a life-threatening allergic reaction characterized by the sudden onset of symptoms such as difficulty breathing, swelling of the throat and tongue, rapid heartbeat, and a drop in blood pressure. […] Skin manifestations are common in systemic mastocytosis, often presenting as: Urticaria pigmentosa: Small, raised patches or spots on the skin that are reddish-brown and can itch when rubbed or scratched.

• #26 Systemic Mastocytosis: Symptoms, Subtypes, Treatment

  https://www.verywellhealth.com/systemic-mastocytosis-8669227

  Systemic mastocytosis is a rare health condition in which too many mast cells (a type of immune system cell) build up in the body, causing allergic reactions and other health issues. This disorder encompasses various subtypes, each presenting unique challenges and symptoms. […] When triggered, mast cells release proteins that start an immune response. This can cause symptoms like itching, flushing, stomach pain, or severe allergic reactions. […] Indolent systemic mastocytosis (ISM) is the most common and typically less severe form of systemic mastocytosis. In indolent systemic mastocytosis, mast cells accumulate in various organs but do not cause significant organ damage or dysfunction. Symptoms may be mild and usually include skin lesions and gastrointestinal issues. […] ASM involves a rapid and extensive accumulation of abnormal mast cells in multiple organs, leading to severe symptoms such as organ dysfunction, bone fractures, and potentially life-threatening complications.

• #27 Systemic Mastocytosis | Jules Bordet Institute

  https://www.bordet.be/en/systemic-mastocytosis

  The signs and symptoms of systemic mastocytosis can include – Flushing, itching. – Abdominal pain, diarrhoea, nausea or vomiting. – Palpitations, feeling faint. – Allergic reactions ranging from urticaria to Quinckes oedema and anaphylaxis – Coughing, shortness of breath, rhinorrhea, lachrymation. – Frequent urination, urinary burning. – Bone and muscle pain. – Depression, mood changes or problems concentrating. – Anaemia or bleeding disorders. – Enlarged liver, spleen or lymph nodes. […] The various triggers for mastocytosis symptoms include: – Insect bites. – Food rich in histamine or histamine liberators. – Physical factors (change in temperature, fever) – Surgery, traumas. – Certain medicines.

• #28 Rare diseases | Systemic Mastocytosis

  https://www.istitutogentili.com/en/rare-diseases/systemic-mastocytosis/

  Systemic mastocytosis (SM) is a rare disease resulting from the clonal expansion and accumulation of abnormal mast cells in various organs and tissues throughout the body. It is a complex and invalidating disease that has a negative impact on the Patients quality of life. […] There are 3 types of symptoms that can present in a Patient with mastocytosis and they depend, at least in part, on the form of the disease in question. […] Mediator-release symptoms: flushing, urticaria, episodes of hypotension/anaphylaxis, headache, nausea, vomiting and diarrhoea, abdominal cramps, adverse reactions to food (e.g. peanuts, walnuts, hazelnuts), adverse drug reactions (most commonly to -lactam antibiotics, NSAIDs and anaesthetics), adverse reactions to certain insect (Hymenoptera) bites, severe osteoporosis.

• #29 How is Systemic Mastocytosis Staged and Classified – HealthTree for Systemic Mastocytosis

  https://healthtree.org/mastocytosis/community/how-is-systemic-mastocytosis-staged-and-classified

  Systemic mastocytosis (SM) is a disorder characterized by an abnormal accumulation of mast cells in one or more organ system. The staging of systemic mastocytosis is based on the extent of organ involvement and the presence of associated hematologic non-mast cell lineage disease (AHNMD). The stages include: […] Patients with ISM have symptoms related to mast cell mediator release and/or infiltration, but they do not have organ damage. […] Patients with SSM have a high burden of mast cells in their bone marrow and other organs, and they may have signs of organ damage. […] Patients with ASM have organ damage caused by the infiltration of mast cells into various organ systems. […] The phases of the disease are typically defined by the stage of the disease, with the early phase characterized by ISM, the intermediate phase by SSM and SM-AHNMD, and the late phase by ASM and MCL. The progression of the disease is usually slow, but it can be rapid in some cases, particularly in patients with ASM or MCL.

• #30 Mastocytosis – The UK Mastocytosis Support Group

  https://ukmasto.org/about-mcd/mastocytosis/

  Mastocytosis is a disorder found in both children and adults which results from too many mast cells in the body. […] Mastocytosis is caused in most adult patients by a genetic change in a protein called KIT on the mast cell which results in too many accumulating in different parts of the body. Increased numbers of mast cells show as pink or dark marks that may itch. […] Bone Marrow Biopsies are performed to confirm a diagnosis of systemic mastocytosis, and to understand which form of the disease is present and track progression. […] Indolent Systemic Mastocytosis (ISM) is the most common form, with perhaps 90% of adults having this type. ISM may remain unchanged through life or, very uncommonly (1-3%), progress to one of the other types mentioned below. Skin lesions are common. […] Smouldering Mastocytosis is a recently agreed upon diagnosis, made when there is higher burden of mast cells (mast cell infiltration of greater than 30% in the bone marrow, serum tryptase over 200 ng/ml and liver or spleen enlarged).

• #31 Indolent Systemic Mastocytosis Symptoms: Their Effects

  https://www.verywellhealth.com/indolent-systemic-mastocytosis-symptoms-8671769

  Indolent systemic mastocytosis symptoms start in different ways, depending on where in the body the excessive amounts of abnormal mast cells are. […] Indolent systemic mastocytosis symptoms start slowly. They can often begin as problems that seem unrelated or mimic other conditions that include: Frequent diarrhea and stomachaches that resemble any general gastrointestinal disorder. Symptoms that appear to be an unexplained allergic reaction, that occur due to a wide range of triggers, or that suddenly change from neutral to intense. Long-lasting facial flushing and heartburn that resembles a food sensitivity. […] Symptoms of indolent systemic mastocytosis can vary widely. While skin symptoms like urticaria pigmentosa can remain chronic, many symptoms spontaneously occur and go away in reaction to triggers. It is possible to experience episodes of severe symptoms that worsen when the mast cells release excess amounts of histamine.

• #32 Orphanet: Smoldering systemic mastocytosis

  https://www.orpha.net/en/disease/detail/158775

  A rare, slowly progressive form of systemic mastocytosis (SM) characterized by gradual accumulation of neoplastic mast cells in the visceral organs. Patients typically present with splenomegaly, hypercellular marrow and, in most cases, urticaria pigmentosa-like skin lesions. […] The age of onset of smoldering systemic mastocytosis (SSM) is in adulthood, with patients tending to be slightly older than those with isolated SM (ISM). The disease is defined by the presence of at least two B-findings, indicative of a high mast cell (MC) burden, and no C findings (organ dysfunction). There is a pronounced MC infiltration (30% in bone marrow (BM) biopsy), organomegaly and tryptase levels above 200 ng/ml. The clinical course is characterized by slow progression without signs of aggressive disease or an associated hematologic neoplasm (AHN). Patients may remain stable for years or may progress into a more advanced variant (aggressive SM (ASM), mast cell leukemia (MCL) or SM with an AHN).

• #33 Orphanet: Aggressive systemic mastocytosis

  https://www.orpha.net/en/disease/detail/98850

  Aggressive systemic mastocytosis (ASM) may occur at any age, occurring predominantly in adults and very rarely in pediatric patients. Presentation is with organ dysfunction related to mast cell invasion (C-findings) and the intense release of mediators including syncope, recurrent flushing, diarrhea, pain, organomegaly. […] The most serious complications include potentially fatal anaphylactic shock and transformation to mast cell leukemia (MCL). Disease progression can be either slow or rapid. In patients with rapid progression, serum tryptase levels increase rapidly, multi-organ damage occurs (or worsens) within a short time, and rapid progression to MCL is frequent. […] The prognosis is usually poor with a median survival of 2 to 4 years. Due to rapid progression to MCL, ASM patients with a MC count in the BM smears 5% have a worse outcome with reduced survival and progression-free survival compared to patients with SRSF2, ASXL1, RUNX1, is associated with progression and poor outcome.

• #34 Systemic mastocytosis: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/systemic-mastocytosis/

  The severe types include aggressive systemic mastocytosis, systemic mastocytosis with an associated hematologic neoplasm, and mast cell leukemia. These types are associated with a reduced life span, which varies among the types and affected individuals. In addition to the general signs and symptoms of systemic mastocytosis, these types typically involve impaired function of an organ, such as the liver, spleen, or lymph nodes. The organ dysfunction can result in an abnormal buildup of fluid in the abdominal cavity (ascites). Aggressive systemic mastocytosis is associated with a loss of bone tissue (osteoporosis and osteopenia) and multiple bone fractures. Systemic mastocytosis with an associated hematologic neoplasm and mast cell leukemia both involve blood cell disorders or blood cell cancer (leukemia). Mast cell leukemia is the rarest and most severe type of systemic mastocytosis. […] Individuals with the milder forms of the condition generally have a normal or near normal life expectancy, while those with the more severe forms typically survive months or a few years after diagnosis.

• #35 Systemic Mastocytosis

  https://www.aaaai.org/conditions-treatments/related-conditions/systemic-mastocytosis

  Aggressive systemic mastocytosis patients have more significant symptoms, including enlarged liver and lymph nodes, as well as blood abnormalities such as anemia and low platelets. Patients with systemic mastocytosis with an associated hematologic neoplasm suffer from additional problems caused by the associated blood malignancy. Mast cell leukemia is very rare, but comes with a difficult prognosis and shorter life span.

• #36 Systemic Mastocytosis: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/203948-overview

  The prognosis in patients with systemic mastocytosis (systemic mast cell disease) is variable. Several prognostic models for systemic mastocytosis have been developed, but there is no consensus regarding the preferred approach for defining prognosis. Young children and patients who present with primarily cutaneous and flushing manifestations tend to have little or no progression of the disease over a considerable length of time. However, older patients and those with more extensive systemic disease involving organ systems other than the skin have a poorer prognosis; although their median duration of survival is not known, it appears to be a few years. […] Early evolution into acute leukemia may occur in as many as 32% of patients with aggressive mastocytosis. Leukemic transformation is rare with indolent systemic mastocytosis.

• #37 Review and Updates on Systemic Mastocytosis and Related Entities

  https://www.mdpi.com/2072-6694/15/23/5626

  Mastocytosis is a rare clonal hematopoietic disorder characterized by proliferation of neoplastic MCs in different organs. […] The heterogeneous clinical presentation of SM is related to MC-released mediators, MC burden, and associated hematological disorders. […] Constitutional symptoms, when present, may include weight loss, pain, nausea, headache, malaise, or fatigue. […] Mediator-associated symptoms occur both in indolent and advanced SM, triggered by factors like stress, allergens, medications, and more. […] Extensive involvement with neoplastic MCs is associated with organ dysfunction. […] Clinical signs and symptoms are classified as B-findings (burden of disease) and C-findings (cytoreduction requiring), which are important factors for establishing an accurate subclassification for SM.

• #38 Indolent Systemic Mastocytosis Symptoms: Their Effects

  https://www.verywellhealth.com/indolent-systemic-mastocytosis-symptoms-8671769

  As a medical term, „indolent” describes a condition that progresses slowly and does not present an immediate threat. Symptoms of indolent systemic mastocytosis, therefore, move gradually. ISM rarely worsens to a more advanced form. […] It also is rare for symptoms of ISM rarely pose an immediate threat or interfere with organ function. Indolent systemic mastocytosis treatment typically involves treating symptoms and reducing the risk of triggers. People with ISM often have low morbidity (chance of becoming ill) and a normal life expectancy, similar to their peers.

• #39 Orphanet: Smoldering systemic mastocytosis

  https://www.orpha.net/en/disease/detail/158775

  Some SSM patients may remain stable for years, while others may progress to more advanced variants of the disease (ASM or MCL), with a poorer prognosis. In general, the prognosis of SSM regarding progression-free survival and overall survival is better than that of ASM or MCL, but poorer than in typical ISM.

• #40 Systemic Mastocytosis – MD Searchlight

  https://mdsearchlight.com/health/systemic-mastocytosis/

  Lymphoid tissues and spleen: The lymph nodes and spleen often are affected in all types of systemic mastocytosis. Lymph nodes enlargement can occur in 20 to 60% of patients with a mild form of the disease, and in most patients with severe forms of the disease. Enlarged spleen can be seen in half of patients with the mild form, and in over 70% of those with severe forms of systemic mastocytosis. […] The type of systemic mastocytosis a person has is one of the most important factors in predicting how the disease will progress. Systemic mastocytosis is a condition where mast cells, a type of white blood cell, build up in different parts of the body like skin, bone marrow and internal organs. Some characteristics of this disease can increase the risk of death due to the disease getting worse, no matter which type of systemic mastocytosis a patient has. These characteristics include:

• #41 Systemic Mastocytosis – MD Searchlight

  https://mdsearchlight.com/health/systemic-mastocytosis/

  Low platelet count: Platelets are small blood cells that help stop bleeding. A count less than 150 10/l indicates a low level. […] Low level of serum albumin: Albumin is a protein in your blood. If the level falls under 35 g/L, this is considered low and could indicate a problem. […] Low hemoglobin levels: Hemoglobin is the part of your red blood cells that carries oxygen. If you have less than 100 g/L or require a transfusion of red blood cells, your levels are considered low. […] High level of lactate dehydrogenase (LDH): LDH is an enzyme found in many body tissues. An elevated level can indicate tissue damage. […] Older age at the start of systemic symptoms: Those who begin showing symptoms over the age of 60 have a higher risk. […] High level of alkaline phosphatase: This is an enzyme found in your liver, bones, and other tissues. A high level could indicate disease.

• #42 Systemic Mastocytosis – MD Searchlight

  https://mdsearchlight.com/health/systemic-mastocytosis/

  Enlargement of the liver and spleen, also known as hepatosplenomegaly. […] Ascites, which is the build-up of fluid in the abdominal cavity. […] Too many immature white blood cells, called blasts, in the bone marrow. […] Weight loss […] If you have a mutation, or change, in a specific gene called ASXL1, you could have a higher risk.

• #43 Systemic Mastocytosis: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/24386-systemic-mastocytosis

  People with systemic mastocytosis may develop raised spots or rashes on their skin. When touched or stroked, the area of skin affected may turn red and start to itch or swell. […] Systemic mastocytosis symptoms may include: Anemia, Bone pain, Excessive bleeding, Fatigue, Flushed skin, Heart palpitations, Hives, Itchy skin, Mood changes or depression, Urticaria pigmentosa (dark, itchy bumps on your skin). […] People with systemic mastocytosis may suddenly develop several symptoms all at once. Healthcare providers may refer to this as a mastocytosis attack or flare. […] Theres no cure for systemic mastocytosis. But healthcare providers have treatment strategies to manage symptoms and complications. […] Those with more aggressive types may have shorter lifespans.

• #44 Mastocytosis Diagnosis & Treatment – NYC | Herbert Irving Comprehensive Cancer Center (HICCC) – New York

  https://www.cancer.columbia.edu/cancer-types-care/types/rare-blood-disorders/conditions/mastocytosis

  Patients with systemic mastocytosis sometimes have „attacks,” when more than one symptom appears at the same time. Afterward they may feel tired and lethargic, complaining of drowsiness and low energy. […] Systemic mastocytosis can take three forms: The most common indolent systemic mastocytosis is a condition that progresses slowly, The second most common form is associated with a disruption of the bloodmaking (hematopoetic) process, The third form aggressive systemic mastocytosis progresses very quickly and can lead to organ damage.

• #45 About Systemic Mastocytosis | Blueprint Medicines

  https://itssmthing.com/about-sm.php

  People living with Non-Advanced SM can experience symptoms such as skin rashes and gut symptoms. People with Advanced SM may experience more severe consequences of mast cell accumulation and activation across the body. This may include anemia, low platelet counts, fluid buildup, bone fractures, enlarged spleen and lymph nodes and, in some cases, organ failure. […] People living with SM may experience a variety of symptoms in different parts of the body. The unpredictable and continual nature of these symptoms can take a toll on people with SM. Its not uncommon for people to report living with SM as disabling.

• #46 How persons with systemic mastocytosis describe the time between symptom onset and receiving diagnosis | Primary Health Care Research & Development | Cambridge Core

  https://www.cambridge.org/core/journals/primary-health-care-research-and-development/article/how-persons-with-systemic-mastocytosis-describe-the-time-between-symptom-onset-and-receiving-diagnosis/DEDD41F33697B668404799755237327F

  The aim of the study was to explore how persons with systemic mastocytosis (SM) described the time between the onset of symptoms and signs and getting the diagnosis. […] The time between symptom and signs onset and diagnosis was perceived as difficult. SM often had a complex and unpredictable effect on a person’s daily life, long before diagnosis. […] Having symptoms and signs with an unknown cause included the participants’ descriptions of numerous symptoms and signs, often years before diagnosis. These could be severe and result in worries for both participants and their next-of-kin. […] The symptoms and signs can include, e.g., skin and gastrointestinal (GI) problems, cognitive disabilities, depression and life-threatening anaphylactic reactions. […] More than 4 of 10 patients have experienced anaphylactic reactions presenting as respiratory problems, low blood pressure, seizures, flush and skin reactions.

• #47 Systemic mastocytosis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/systemic-mastocytosis/diagnosis-treatment/drc-20450478

  Your doctor may recommend medications to treat symptoms, for example, with antihistamines. […] If you have aggressive systemic mastocytosis, systemic mastocytosis associated with another blood disorder or mast cell leukemia, you may be treated with chemotherapy medications to reduce the number of mast cells. […] Your doctor regularly monitors the status of your condition using blood and urine samples. […] Regular bone density measurements can monitor you for problems such as osteoporosis.

• #48 Systemic Mastocytosis: Causes, Symptoms, Diagnosis, and Treatment

  https://www.webmd.com/allergies/systemic-mastocytosis

  The more serious types, such as ASM and MCL, move more rapidly. For ASM, you may need treatment for problems such as bone loss. If you have MCL, which is a rare type, you may get blood disorders, including leukemia. […] Whatever type you have, after your diagnosis, you’ll go to regular follow-up appointments with your doctor. They’ll track your disease and check that your treatments are working.

• #49 Systemic mastocytosis | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/systemic-mastocytosis

  Complications of systemic mastocytosis can include: Anaphylactic reaction, Blood disorders, Peptic ulcer disease, Reduced bone density, Organ failure. […] Systemic mastocytosis most commonly occurs in adults. Another type of mastocytosis, cutaneous mastocytosis, typically occurs in children and usually affects only the skin. It generally doesn’t progress to systemic mastocytosis. […] Treatment may vary, depending on the type of systemic mastocytosis and the body organs affected. Treatment generally includes controlling symptoms, treating the disease and regular monitoring. […] Identifying and avoiding factors that may trigger your mast cells, such as certain foods, medications or insect stings, can help keep your systemic mastocytosis symptoms under control. […] Your doctor regularly monitors the status of your condition using blood and urine samples. You may be able to use a special home kit to collect blood and urine samples while you’re experiencing symptoms, which gives your doctor a better picture of how systemic mastocytosis affects your body. Regular bone density measurements can monitor you for problems such as osteoporosis.

• #50 Systemic mastocytosis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/systemic-mastocytosis/diagnosis-treatment/drc-20450478

  Systemic mastocytosis most commonly occurs in adults. […] This is the most common type and usually doesn’t include organ dysfunction. Skin symptoms are common, but other organs may be affected, and the disease may worsen slowly over time. […] This type is associated with more-significant symptoms and may include organ dysfunction and worsening disease over time. […] This severe type develops rapidly and is often associated with organ dysfunction and damage. […] This rare type is more severe, with significant symptoms, and is usually associated with progressive organ dysfunction and damage. […] Systemic mastocytosis can be a complex disease. Understanding your condition is critical to ongoing care and prevention of complications. […] Identifying and avoiding factors that may trigger your mast cells, such as certain foods, medications or insect stings, can help keep your systemic mastocytosis symptoms under control.

• #51 Review and Updates on Systemic Mastocytosis and Related Entities

  https://www.mdpi.com/2072-6694/15/23/5626

  The diagnosis of SM requires the synthesis of BM morphologic, immunophenotypic, and molecular findings (KIT mutation), as well as clinical signs and symptoms (B-findings and C-findings). […] Indolent SM, although producing morbidity, is associated with normal OS. In contrast, patients with advanced SM typically have a much-shortened life expectancy in addition to a decreased quality of life. […] The classical driver KIT D816V mutation is a hallmark of and minor diagnostic criterion for SM; other KIT mutations have also been reported including germline KIT mutations associated with familial mastocytosis. […] The complex integrated diagnostic process for SM is complicated by pitfalls and requires expertise to avoid misdiagnosis.

• #52 Mastocytosis and Mast Cell Activation Syndrome – Immunology; Allergic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/immunology-allergic-disorders/allergic-autoimmune-and-other-hypersensitivity-disorders/mastocytosis-and-mast-cell-activation-syndrome

  The baseline level of tryptase is elevated in systemic mastocytosis but is typically normal in cutaneous mastocytosis and mast cell activation syndrome. […] All patients with systemic mastocytosis should be treated with H1 and H2 blockers and should carry a prefilled, self-injecting epinephrine syringe. […] For systemic mastocytosis, use H1 and H2 blockers and sometimes cromolyn, and for aggressive mastocytosis, consider midostaurin, avapritinib, interferon alfa-2b, systemic corticosteroids.

• #53 Discover SM Home | Information for HCPs

  https://www.systemicmastocytosis-hcp.com/

  Recognising common symptoms is just the beginning. Systemic mastocytosis (SM) can present in patients as a range of unpredictable and debilitating symptoms across multiple organ systems from skin lesions, anaphylaxis and diarrhoea, to organ damage. […] Early diagnosis of disease and active management of life-threatening symptoms, such as anaphylaxis, are critical. […] Anaphylaxis, a result of mast cell mediator release, is an acute and suddenly occurring systemic hypersensitivity reaction in multiple organ systems. In severe episodes, patients may experience palpitations, dizziness and reduced blood pressure due to vasodilation.

• #54 Mastocytosis – Wikipedia

  https://en.wikipedia.org/wiki/Mastocytosis

  Systemic mastocytosis involves the bone marrow in the majority of cases and in some cases other internal organs, usually in addition to involving the skin. Mast cells collect in various tissues and can affect organs where mast cells do not normally inhabit such as the liver, spleen and lymph nodes, and organs which have normal populations but where numbers are increased. In the bowel, it may manifest as mastocytic enterocolitis. […] Patients with indolent systemic mastocytosis have a normal life expectancy. The prognosis for patients with advanced systemic mastocytosis differs depending on type of disease with MCL being the most serious form with short survival.

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