Materiały źródłowe

• #1 Mastocytosis — DermNet

  https://dermnetnz.org/topics/mastocytosis

  There is no known way to prevent mastocytosis. […] Avoidance of triggers for histamine release/ mast cell degranulation is important in patients with symptomatic mastocytosis. […] Special care should be taken with patients with diffuse cutaneous or systemic mastocytosis, especially if they have elevated serum tryptase, prior to receiving anaesthetic or having imaging that requires radio-contrast.

• #2 Mastocytosis: Symptoms and Treatment | Rencic Dermatology

  https://www.rencicderm.com/conditions/mastocytosis

  Preventing mastocytosis isn’t currently possible due to its primarily genetic origin, involving mutations affecting mast cells. […] However, individuals can take proactive steps to manage their symptoms and enhance their quality of life. This involves identifying and avoiding triggers that can worsen mast cell activation and symptoms, such as certain foods, medications, environmental factors, and emotional stressors. […] Collaborate closely with your healthcare providers to develop personalized medication regimens tailored to manage symptoms like itching, flushing, and gastrointestinal issues. […] Wear medical identification, such as bracelets or necklaces, indicating mastocytosis status, especially if you’re at risk of severe allergic reactions or anaphylaxis, to ensure prompt and appropriate medical attention during emergencies.

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

  https://www.everydayhealth.com/rare-diseases/systemic-mastocytosis/

  There is no known way to prevent the genetic mutations that cause mastocytosis. […] The most meaningful lifestyle change for systemic mastocytosis is avoiding the triggers that can lead to inflammation and organ damage. This is usually more important in people with indolent or smoldering SM than the more advanced forms of SM, says Gotlib. […] Triggers can include: Rubbing or friction on your skin, Exercise and physical activity, Venom from bites and stings from fire ants, wasps, bees, jellyfish, snakes, fleas, and spiders, Alcohol, Spicy foods, Certain medications, including opioids, nonsteroidal anti-inflammatory drugs (NSAIDs), some local anesthetics, and contrast dyes, Sudden changes in temperature, Stress, whether emotional, physical (including pain), or environmental, Sunlight, Infections (viral, bacterial or fungal).

• #4 Treatment of Indolent and Advanced Systemic Mastocytosis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9084172/

  Management of Indolent and Smoldering SM is focused on preventing anaphylactic reactions and identifying and avoiding symptom triggers. […] The first approach is to identify symptom triggers and suggest avoidance strategies of triggers, such as physical stimuli (heat, change of temperature, pressure, cold, rubbing), exercise, sleep deprivation, emotions, drugs (opiates, contrast media, succinylcholine, nonsteroidal anti-inflammatory drugs, agents with tetrahydroisoquinoline such as quinolones, atracurium, and rocuronium), alcohol, food, and Hymenoptera stings. […] All patients should be prescribed self-injectable epinephrine and should be trained to treat attacks. […] Prevention of new episodes is based on chronic treatment with H1- and H2-antihistamines and leukotriene antagonists, while oral corticosteroids (prednisone 0.51 mg/kg/day) should be proposed only in very resistant patients.

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

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

  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. […] Systemic mastocytosis can be a complex disease. Understanding your condition is critical to ongoing care and prevention of complications. […] Here are some self-care tips: Identify and avoid your triggers. […] Follow your doctor’s recommended care and ongoing monitoring. […] Wear a medical alert bracelet or necklace or carry a medical card identifying that you have systemic mastocytosis. Ask your doctor to give you information for health care professionals that you can carry with you in case you need emergency care.

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

  https://www.everydayhealth.com/rare-diseases/systemic-mastocytosis/

  There is no known way to prevent the genetic mutations that cause mastocytosis. […] The most meaningful lifestyle change for systemic mastocytosis is avoiding the triggers that can lead to inflammation and organ damage. This is usually more important in people with indolent or smoldering SM than the more advanced forms of SM, says Gotlib. […] Triggers can include: Rubbing or friction on your skin, Exercise and physical activity, Venom from bites and stings from fire ants, wasps, bees, jellyfish, snakes, fleas, and spiders, Alcohol, Spicy foods, Certain medications, including opioids, nonsteroidal anti-inflammatory drugs (NSAIDs), some local anesthetics, and contrast dyes, Sudden changes in temperature, Stress, whether emotional, physical (including pain), or environmental, Sunlight, Infections (viral, bacterial or fungal).

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

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

  Unfortunately, there is no known way to prevent mastocytosis since gene mutations associated with the condition often develop spontaneously. For people with mastocytosis, the best way to reduce the severity of your symptoms is to identify and avoid anything that might trigger mast cell activation and histamine release. […] Triggers vary from person to person and may include: […] Extreme temperatures […] Certain medications (e.g., non-steroidal anti-inflammatory drugs) […] Alcohol […] Spicy foods […] Stress […] Allergens (e.g., dust, pollen, insect bites)

• #8 Cutaneous and Systemic Manifestations of Mastocytosis | AAFP

  https://www.aafp.org/pubs/afp/issues/1999/0601/p3047.html

  Patients with symptomatic mastocytosis should identify and avoid triggering factors. Extremes of temperature, alcohol and certain drugs should be avoided completely or used with caution. […] The mainstay of therapy is histamine H1 and H2 blockers and the avoidance of triggering factors. […] Treatment is initiated in the hospital, and should begin at a low dosage. The patient should receive antihistamines before intervention with aspirin is undertaken. […] Both H1 and H2 blockers should be used to treat anaphylaxis. […] Patients with mastocytosis should also wear a medical alert bracelet and carry an adrenaline-filled syringe. […] The emergency treatment of shock in association with mastocytosis is the same as that of anaphylaxis.

• #9

  https://link.springer.com/article/10.1007/s40521-023-00349-2

  Mastocytosis is associated with a high risk of anaphylaxis, in part due to drug hypersensitivity reactions (DHR). […] To protect patients against DHR and anaphylaxis, general avoidance of potential drug triggers is common practice, which often deprives patients of important drugs at times of need and limits their options. […] Recent data shows that general avoidance of drugs with potential mast cell activation action is not indicated in all patients with mastocytosis, but guidelines are lacking. […] Drugs tolerated before and after the onset of mastocytosis should not be avoided and a personalized approach is recommended to address drugs inducing mast cell activation. […] Pre-medication (RCM, local and general anesthetics, vaccines), use of safer alternatives (opioids, NBMAs, NSAIDs in selected cases), and drug challenges (NSAIDs in most cases) are recommended to increase the safety of patients with mastocytosis when introduced to new drugs.

• #10

  https://link.springer.com/article/10.1007/s40521-023-00349-2

  Avoidance should be restricted to specific drugs to which there is a history of reactions. […] Pre-medications should be used judiciously before tests and procedures known to induce reactions. […] Pre-emptive and empirical avoidance of drugs is not recommended in children with mastocytosis who have not presented previous reactions or have not been exposed to the drug.

• #11

  https://www.aaaai.org/allergist-resources/ask-the-expert/answers/2022/prophylaxis

  The goal of peri-operative treatment in mastocytosis is the prevention of mast cell mediator signs and symptoms. […] Effective premedication for surgery in systemic mastocytosis patients is given 1 hour prior to the surgery with H1 and H2 antihistamines and benzodiazepines. […] To blunt the mast cell response, utilizing cromolyn sodium as a mast cell stabilizer could help as well. […] Therefore, the best way to avoid mast cell degranulation in mastocytosis is to avoid known triggers that have precipitated prior episodes and potential triggers, including pharmacological, psychological and mechanical factors, and temperature changes. […] Any regular medications used to maintain mast cell stability like cromolyn sodium and limit the effects of mast cell mediators should be continued.

• #12 Mastocytosis Treatment & Management: Medical Care, Consultations, Diet

  https://emedicine.medscape.com/article/1057932-treatment

  Medical alert bracelets should be made available, and an epinephrine self-injector demonstration should be performed and self-injector prescribed for patients with systemic mastocytosis or patients with episodes of vascular collapse. […] Patients with recurrent episodes of vascular collapse may be prophylactically placed on H1 and H2 antihistamines to lessen the severity of attacks. […] Premedication with antihistamines and corticosteroids is recommended before such procedures in patients at risk.

• #13 Update on Mastocytosis (Part 2): Categories, Prognosis, and Treatment | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/en-update-on-mastocytosis-part-2–articulo-S157821901500311X?xhy=Dr56DrLjUdaMjzAgze452SzSInMN&rfr=truhgiz&y=kEzTXsahn8atJufRpNPuIGh67s1

  In addition to general measures, all patients should be administered treatment aimed at controlling symptoms due to mast cell mediator release in accordance with the type and severity of symptoms they typically experience. […] Different combinations of drugs are indicated depending on the patient’s clinical manifestations.

• #14 Long-Term Successful Treatment of Indolent Systemic Mastocytosis With Omalizumab | MDedge

  https://mdedge.com/fedprac/article/234325/rare-diseases

  This case study suggests that omalizumab may help prevent anaphylaxis and reduce disease burden associated with systemic mastocytosis, but further studies and formal clinical trials are needed to confirm these findings. […] Although not approved by the FDA for the treatment of SM, omalizumab may aid in the prevention of anaphylaxis, the reduction of disease burden, and the improvement in quality of life for patients with SM.

• #15 Treatment of Indolent and Advanced Systemic Mastocytosis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9084172/

  VIT is mandatory since it is the only life-saving treatment for these patients, and it should be prolonged long-life with a 34 month-interval, according to European and American guidelines. […] Mastocytosis patients are at higher risk of reactions during build-up and maintenance phases, but some authors recently observed no adverse events in 8 patients undergoing 12 ultra-rush VIT, both in the build-up and maintenance phases. […] Nevertheless, because of severe reactions, expert personnel should carry out an ultra-rush protocol with the prompt availability of resuscitation equipment.

• #16 Systemic Mastocytosis: Symptoms, Causes & Treatment

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

  Systemic mastocytosis happens when you have the genetic change that causes it. That means you cant prevent it. But you can reduce your risk for attacks or flares by avoiding triggers. […] Knowing how to avoid triggers is a big part of living with systemic mastocytosis. If you have this condition, you should: Always carry an EpiPen to treat severe, life-threatening allergic reactions. Know your triggers and do your best to avoid them. Manage stress by practicing meditation or other forms of mindfulness. Wear a medical alert bracelet that lists medications you cant take.

• #17 Mastocytosis: Types, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/5908-mastocytosis

  No, it cant. Mastocytosis happens when KIT genes mutate, creating abnormal mast cells that multiply uncontrollably. If you have mastocytosis, you can reduce your risk of allergic reactions by knowing what triggers your allergic reactions. […] If certain medications trigger the condition, consider wearing a medical alert bracelet with information that lets other people know what medications you shouldnt receive. […] If you have systemic mastocytosis, you should always carry a self-injecting syringe of epinephrine, a hormone made by adrenal glands. Epinephrine treats severe allergic reactions that could become life-threatening anaphylactic shock.

• #18 Mastocytosis: Symptoms and Treatment | Rencic Dermatology

  https://www.rencicderm.com/conditions/mastocytosis

  Regularly monitor your symptoms and their triggers through journaling or diaries to identify patterns and develop effective management strategies. […] Stay informed about the latest research and developments in mastocytosis management and seek support from patient communities for valuable insights and support. […] While mastocytosis cannot be prevented, you can empower yourself to lead a fulfilling life while effectively managing your symptoms and minimizing their impact on your overall well-being with guidance and recommendations from knowledgeable healthcare providers.

• #19 Update on Mastocytosis (Part 2): Categories, Prognosis, and Treatment | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/en-update-on-mastocytosis-part-2–articulo-S157821901500311X?xhy=Dr56DrLjUdaMjzAgze452SzSInMN&rfr=truhgiz&y=kEzTXsahn8atJufRpNPuIGh67s1

  Physical measures can have a very important impact on reducing mast cell activation and the need for pharmacologic treatment. […] These measures include avoidance of sudden changes in temperature, exposure to heat, and rubbing of lesions. […] As no cures are currently available for mastocytosis and the prognosis is generally favorable, the goal of treatment is symptomatic relief in most cases. […] Treatment recommendations include general measures aimed at preventing factors that can trigger massive mast cell degranulation and more specific measures to control symptoms caused by the acute or chronic release of mast cell mediators; cytoreductive therapy is also recommended in aggressive mastocytosis to reduce the mast cell burden. […] Diffuse cutaneous mastocytosis, which is very rare, should be considered a true medical emergency due to the high risk of widespread mast cell release, and patients require admission to specialized units experienced in this condition and the use of full-dose treatments, including phototherapy. […] Treatment with chemotherapeutic agents such as interferon and tyrosine kinase inhibitors, which modulate biologic response, is formally contraindicated in children with mastocytosis, except in life-threatening situations.

• #20 Update on Mastocytosis (Part 2): Categories, Prognosis, and Treatment | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/en-update-on-mastocytosis-part-2–articulo-S157821901500311X?xhy=Dr56DrLjUdaMjzAgze452SzSInMN&rfr=truhgiz&y=kEzTXsahn8atJufRpNPuIGh67s1

  Mastocytosis is a term used to describe a heterogeneous group of disorders characterized by clonal proliferation of mast cells in different organs. […] The goal of treatment should be to control clinical manifestations caused by the release of mast cell mediators and, in more aggressive forms of the disease, to reduce mast cell burden. […] As in other rare or uncommon diseases, the management of mastocytosis should involve multidisciplinary teams and contact with specialized reference centers. […] All patients and/or carers should be provided with written information on the disease, including general information on factors that can trigger symptoms due to the release of mast cell mediators, and personalized information adapted to the patient’s history of tolerance of these triggers.

• #21 Mastocytosis – Knowledge @ AMBOSS

  https://www.amboss.com/us/knowledge/mastocytosis/

  Treatment is specialist-guided and includes: […] Prevention of anaphylaxis: e.g., H1 antihistamines and avoidance of triggers of mast cell degranulation (e.g., alcohol consumption, aspirin) […] Avoidance of triggers of mast cell degranulation.

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

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

  One of the best ways to manage systemic mastocytosis is to avoid triggers. You’ll want to talk to your doctor and keep track of all the things that cause your symptoms, such as stress or spicy food. […] Because the disease can cause a life-threatening allergic reaction, you’ll also want to carry an epinephrine auto-injector at all times.

• #23 Mastocytosis – Australasian Society of Clinical Immunology and Allergy (ASCIA)

  https://www.allergy.org.au/patients/allergy-testing/mastocytosis

  People who have frequent reactions as a result of mastocytosis or mast cell activation syndromes need ongoing management by a doctor, which should include: […] Strategies to avoid triggers if known.

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