Materiały źródłowe

• #1

  https://link.springer.com/article/10.1007/s00277-002-0575-z

  Mastocytosis consists of a group of disorders characterized by a pathologic increase in mast cells in tissues including skin, bone marrow, liver, spleen, and lymph nodes. […] Most patients experience symptoms related to mast cell mediator release, and prevention of the effects of these mediators on tissues constitutes the major therapeutic goal in the management of mastocytosis. […] Management of patients within all categories of mastocytosis includes: (1) a careful counseling of patients (parents in pediatric cases) and care providers, (2) avoidance of factors triggering acute mediator release, (3) treatment of acute mast cell mediator release, (4) treatment of chronic mast cell mediator release, and if indicated (5) an attempt to treat organ infiltration by mast cells.

• #2 Mastocytosis: What It Looks Like, Causes, and Symptoms

  https://www.verywellhealth.com/mastocytosis-7566905

  You cant prevent mastocytosis because the condition results from a gene mutation that happens before birth. […] However, you can help control your symptoms. The best way to do that is by avoiding triggers. This takes time and practice to learn what causes symptoms to flare up. You can also work with your healthcare provider (or treatment team) to develop a treatment plan that helps control your symptoms.

• #3 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.

• #4

  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. […] Recommended pre-operative medications for mastocytosis patients include an H1 antihistamine IV 30 minutes before the procedure, an H2 antihistamine 15 minutes before the procedure and prednisolone 12 and 2 hours before the procedure. […] 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.

• #5

  https://link.springer.com/article/10.1007/s00277-002-0575-z

  Mastocytosis consists of a group of disorders characterized by a pathologic increase in mast cells in tissues including skin, bone marrow, liver, spleen, and lymph nodes. […] Most patients experience symptoms related to mast cell mediator release, and prevention of the effects of these mediators on tissues constitutes the major therapeutic goal in the management of mastocytosis. […] Management of patients within all categories of mastocytosis includes: (1) a careful counseling of patients (parents in pediatric cases) and care providers, (2) avoidance of factors triggering acute mediator release, (3) treatment of acute mast cell mediator release, (4) treatment of chronic mast cell mediator release, and if indicated (5) an attempt to treat organ infiltration by mast cells.

• #6 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.

• #7 Cutaneous mastocytosis treatment: strategies, limitations and perspectives

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6320483/

  The prevention of the anaphylactic reactions include identification and avoidance of trigger factors for mast cells degranulation. In general, common triggers for mast cells degranulation include Hymenoptera sting, drugs (opioids, non-steroidal anti-inflammatory drugs, muscle relaxants, iodinated contrast media), stress, alcohol, spicy foods, heat, cold and exercise. […] With regard to the high risk of anaphylaxis in adult patients with mastocytosis, being equipped with 2 adrenaline autoinjectors of 0.3 mg is recommended. […] The risk of anaphylaxis in childhood-onset mastocytosis is lower than in adults. It is estimated to be 5-10% in children compared with 22-50% in adults with mastocytosis. […] Hence, there is no consensus whether to prescribe an adrenaline autoinjector for all children with CM.

• #8 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).

• #9 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. […] Histamine H1 antagonists are used to treat the pruritus, flushing, bullae and urticaria. […] Histamine H2 blockers can be used to decrease the gastric hyperacidity that often accompanies concomitant ulcer disease in patients with systemic mastocytosis. […] 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. Fluids, adrenaline, antihistamines and pressor agents are often required.

• #10 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.

• #11 DEGRANULAR LIST — MastoKids

  http://www.mastokids.org/degranular-list

  While there is a large variability factor concerning things that cause the mast cells to degranulate in patients with pediatric mastocytosis, many triggers have been found to have a commonality among the general mastocytosis community and as such, it is wise to avoid these factors or to carefully monitor a child when such triggers cannot be avoided. […] Factors in this group should be avoided. If medications are deemed to be essential, medical supervision must be provided and emergency interventions immediately available. […] Factors in this group should be approached carefully, under close supervision by a medically trained person, parent or caregiver who is prepared to administer emergency treatment if necessary. Reactions to these triggers may vary in degree of severity, so caution and supervision are consistently required.

• #12 Cutaneous mastocytosis treatment: strategies, limitations and perspectives

  https://www.termedia.pl/Cutaneous-mastocytosis-treatment-strategies-limitations-and-perspectives,7,33470,1,1.html

  The prevention of the anaphylactic reactions include identification and avoidance of trigger factors for mast cells degranulation. In general, common triggers for mast cells degranulation include Hymenoptera sting, drugs (opioids, non-steroidal anti-inflammatory drugs, muscle relaxants, iodinated contrast media), stress, alcohol, spicy foods, heat, cold and exercise. […] With regard to the high risk of anaphylaxis in adult patients with mastocytosis, being equipped with 2 adrenaline autoinjectors of 0.3 mg is recommended. […] The anesthetic and surgical procedures are the risk factors of mast cell mediator-related symptoms and anaphylaxis in all patients with mastocytosis, however the incidence of perioperative anaphylaxis is low. The surgical procedures and anesthesia should not be avoided in patients with mastocytosis. Nevertheless, mastocytosis patients require prophylactic antimediator therapy (PAT) and adequate anesthetic management. The PAT is based on H1/H2 antihistamines, glucocorticoids and benzodiazepines given 1 h before the anesthesia.

• #13 Cutaneous mastocytosis treatment: strategies, limitations and perspectives

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6320483/

  Surely enough, providing an adrenaline autoinjector is recommended for CM children with an extensive skin involvement, blistering, persistently elevated serum tryptase level and anaphylaxis in medical history. […] The anesthetic and surgical procedures are the risk factors of mast cell mediator-related symptoms and anaphylaxis in all patients with mastocytosis, however the incidence of perioperative anaphylaxis is low. […] Nevertheless, mastocytosis patients require prophylactic antimediator therapy (PAT) and adequate anesthetic management. […] Hymenoptera specific immunotherapy (SIT) is recommended in mastocytosis patients with IgE-mediated anaphylactic reactions to honey bee or wasp or both. SIT in Hymenoptera anaphylaxis is the only available option that reduces the risk of life-threatening episodes and should be performed life-long in patients with mastocytosis.

• #14 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.

• #15

  https://www.mastcellhope.org/education/common-treatments/

  The first step to managing mast cell disease is to adopt a prophylactic approach to all identified known and potential triggers. […] Aspirin therapy should always be initiated and managed under the direct supervision of a physician. […] Emergency treatment for mast cell disease is administered in the case of anaphylactic shock. All patients should create an emergency treatment plan with their physician that includes: Increasing H1 and H2 blockers, Taking Benadryl, Using an inhaler, Using a self-injectable Epi-Pen.

• #16 Treatments for MCAS & Mast Cell Disease | The EDS Clinic

  https://www.eds.clinic/articles/treatment-of-mast-cell-disease

  Both diet and environment can trigger symptoms so an important first step in managing mast cell diseases (MCAD) including Mast Cell Activation Syndrome (MCAS) is to adopt a prophylactic approach to all identified known and likely triggers. […] Aspirin may be prescribed in small doses in combination with histamine blockers to help manage symptoms, such as flushing of the skin and symptoms associated with the excess release of prostaglandin (PG) D2. […] While aspirin is available over-the-counter, aspirin therapy should always be initiated and managed under the direct supervision of a physician. […] Anti-IgE therapy helps with overall mast cell stability and therefore a wide range of symptoms affecting all organ systems.

• #17 Cutaneous and Systemic Manifestations of Mastocytosis | AAFP

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

  Aspirin has been used to treat the flushing that is associated with elevated prostaglandin levels during attacks. Caution is required, however, because of the potential for aspirin and other nonsteroidal anti-inflammatory drugs (NSAIDs) to cause degranulation. Treatment with aspirin is reserved for use in patients with vascular collapse that is not prevented by H1 and H2 blocker prophylaxis. […] Treatment is initiated in the hospital, and should begin at a low dosage. The patient should receive antihistamines before intervention with aspirin is undertaken. […] PUVA therapy may provide cosmetic skin changes and temporary relief from symptoms. […] Systemic steroids may be necessary to control the severe skin disease, malabsorption or ascites of systemic mastocytosis.

• #18 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.

• #19 Perioperative Management – TMS – The Mast Cell Disease Society, IncAccessibilityIncrease TextDecrease TextGrayscaleHigh ContrastNegative ContrastLight BackgroundLinks UnderlineReadable FontReset

  https://tmsforacure.org/perioperative-management/

  While the incidence of hypersensitivity to anesthesia and surgical procedures in patients with mast cell diseases (mastocytosis and MCAS) is unknown, various non-specific triggers in the perioperative setting may cause mast cell degranulation, and thus immediate hypersensitivity. Therefore, the goal of all perioperative management is prevention of mast cell mediator release. […] Measures to prevent triggering mast cell degranulation in adults and children should be utilized whenever possible. […] Prevention also includes perioperative antianxiety medications to avoid precipitating mast cell degranulation; maintenance of a steady environmental temperature throughout the entire surgical experience; minimizing friction and mechanical trauma (i.e. tape, tourniquet use, etc.) near mastocytosis skin lesions; careful positioning of the patient, being mindful of possible osteoporosis or osteolysis; avoiding histamine releasing drugs such as atracurium and mivacurium; pre-treating to prevent nausea and vomiting; aggressive treatment of pain, which is a potent mast cell degranulator, including utilizing some acceptable forms of opioids (i.e. fentanyl); use of H1 and H2 receptor antagonists to maintain mast cell stability. […] With these measures, patients with mast cell diseases can be prepared for surgery with a plan that includes preventing mast cell degranulation by identification of possible triggers, rapid recognition of degranulation when it does occur and immediate appropriate intervention.

• #20 What makes a pregnant woman with mastocytosis a high risk patient? | Actual Gynecology And Obstetrics

  https://www.actualgyn.com/en/article/2020/234

  Mastocytosis represents a group of rare related disorders, with potential for unpredictably heighetned mast cell activity in response to the stress of labour, indicating importance in a management of such condition during pregnancy. […] Mastocytosis is perceived as a medical management dilemma because of its potential for unpredictably heighetned mast cell activity in response to the stress of labour and medications given with the potential for activating the disease. […] There are minimal recommendations in the literature regarding medication use in the management of this disease during pregnancy and labour. […] In general, there is no contraindication for pregnancy if mastocytosis is under appropriate medical control. […] Taking into account potential risk of long-term exposure to salicylates in a patient with mastocytosis, and the importance of preeclampsia prevention, our maternal/fetal medicine consultant recommended low dose aspirin (100 mg daily) from 12th week of gestation.

• #21 What makes a pregnant woman with mastocytosis a high risk patient? | Actual Gynecology And Obstetrics

  https://www.actualgyn.com/en/article/2020/234

  This case highlights an important lessons. Firstly, pregnancy induced changes in the immune system could be potentially harmful in puerperium of pregnant women with mastocytosis. […] In the treatment of pregnant women with mastocytosis ready access to epinephrine, adequate intravenous access and the availability of anesthesia personnel to assist the resuscitation is essential to secure favourable pregnancy outcome.

• #22 Cutaneous mastocytosis treatment: strategies, limitations and perspectives

  https://www.termedia.pl/Cutaneous-mastocytosis-treatment-strategies-limitations-and-perspectives,7,33470,1,1.html

  Hymenoptera specific immunotherapy (SIT) is recommended in mastocytosis patients with IgE-mediated anaphylactic reactions to honey bee or wasp or both. SIT in Hymenoptera anaphylaxis is the only available option that reduces the risk of life-threatening episodes and should be performed life-long in patients with mastocytosis.

• #23

  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. […] Avoidance should be restricted to specific drugs to which there is a history of 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.

• #24 Mastocytosis – USZ

  https://www.usz.ch/en/disease/mastocytosis/

  There is no direct possibility of prevention. But an indirect one: If you know the trigger that causes you to develop symptoms of mastocytosis, you can avoid this triggering stimulus. Perhaps you are hypersensitive to certain foods or medications without knowing it then you should try to identify them and avoid them. […] The better you know them, the better you can try to avoid them. You will probably need patience to find these triggers but it can be worth it. An allergological clarification is often very helpful. It is even possible that you will then live without any symptoms despite mastocytosis.

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