Materiały źródłowe

• #1 Systemic Mastocytosis: Symptoms, Causes & Treatment

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

  Healthcare providers cant cure the condition. But treatment can ease your symptoms. […] Healthcare providers treat systemic mastocytosis by managing symptoms and complications. For example, if you have excess stomach acid, they may prescribe H2 blockers (antacids). If the condition causes anemia, theyll treat the anemia. […] Systemic mastocytosis treatments may include: Antihistamines to relieve itching, flushing and other skin reactions; Corticosteroids to reduce inflammation; Bisphosphonates to help with bone weakness; Targeted therapy, which focuses on the protein in abnormal mast cells; Chemotherapy, if systemic mastocytosis becomes cancerous; Splenectomy to remove an enlarged spleen; Bone marrow transplant to treat aggressive or advanced systemic mastocytosis. […] If you have systemic mastocytosis, you should always carry an EpiPen. Epinephrine treats anaphylactic shock, a severe, life-threatening allergic reaction.

• #2 Systemic Mastocytosis, Version 2.2019, NCCN Clinical Practice Guidelines in Oncology in: Journal of the National Comprehensive Cancer Network Volume 16 Issue 12 (2018)

  https://jnccn.org/view/journals/jnccn/16/12/article-p1500.xml

  Mastocytosis is a group of heterogeneous disorders resulting from the clonal proliferation of abnormal mast cells and their accumulation in the skin and/or in various extracutaneous organs. Systemic mastocytosis is the most common form of mastocytosis diagnosed in adults, characterized by mast cell infiltration of one or more extracutaneous organs (with or without skin involvement). […] The management of patients with mastocytosis requires a multidisciplinary team approach (involving dermatologists, hematologists, gastroenterologists, pathologists, and allergists/immunologists) preferably in specialized medical centers with expertise in the treatment of patients with mast cell disorders. […] These NCCN Guidelines provide recommendations for the diagnosis and management of patients with SM. Management of CM is not included in these guidelines. Referral to centers with expertise in CM is strongly recommended.

• #3 Systemic Mastocytosis | Huntsman Cancer Institute | University of Utah Health

  https://healthcare.utah.edu/huntsmancancerinstitute/hematologic-cancers/systemic-mastocytosis

  Systemic mastocytosis is a rare disorder that happens when too many mast cells build up in parts of your body. […] Systemic mastocytosis originates in the bone marrow, and can happen in the skin, liver, spleen, stomach, small intestine, or colon. […] Your care team may include these specialists: Hematologists (Blood specialist doctors), pain and palliative care doctors, dermatologists, and doctors from other specialties, as needed. […] Our care teams may also include advanced care nurses, and physician assistants. Everyone on your care team talks with each other to plan and give treatment. They can talk to you about any questions or concerns you have about systemic mastocytosis.

• #4 Systemic Mastocytosis: Symptoms, Causes & Treatment

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

  Healthcare providers cant cure the condition. But treatment can ease your symptoms. […] Healthcare providers treat systemic mastocytosis by managing symptoms and complications. For example, if you have excess stomach acid, they may prescribe H2 blockers (antacids). If the condition causes anemia, theyll treat the anemia. […] Systemic mastocytosis treatments may include: Antihistamines to relieve itching, flushing and other skin reactions; Corticosteroids to reduce inflammation; Bisphosphonates to help with bone weakness; Targeted therapy, which focuses on the protein in abnormal mast cells; Chemotherapy, if systemic mastocytosis becomes cancerous; Splenectomy to remove an enlarged spleen; Bone marrow transplant to treat aggressive or advanced systemic mastocytosis. […] If you have systemic mastocytosis, you should always carry an EpiPen. Epinephrine treats anaphylactic shock, a severe, life-threatening allergic reaction.

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

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

  Systemic mastocytosis is a disease where too many abnormal mast cells (a type of white blood cell) build up in your skin and other organs. […] There’s no cure, but treatments can help you manage the condition. […] Consider reaching out to family and friends for support as you manage this disease. They can help you deal with the emotions and stress that come with the challenges of a long-term condition like 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. […] 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. […] If you find that the challenges of managing your disease are making you depressed or anxious, talk to your doctor. They can put you in touch with mental health professionals who are experts in treating these problems.

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

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

  Systemic mastocytosis care at Mayo Clinic […] Our caring team of Mayo Clinic experts can help you with your systemic mastocytosis-related health concerns Start Here […] 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. […] A health care professional can teach you how to give yourself an epinephrine injection in the event that you have a severe allergic response when your mast cells are triggered. […] 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.

• #7 Systemic Mastocytosis, Version 2.2019, NCCN Clinical Practice Guidelines in Oncology in: Journal of the National Comprehensive Cancer Network Volume 16 Issue 12 (2018)

  https://jnccn.org/view/journals/jnccn/16/12/article-p1500.xml

  Mastocytosis is a group of heterogeneous disorders resulting from the clonal proliferation of abnormal mast cells and their accumulation in the skin and/or in various extracutaneous organs. Systemic mastocytosis is the most common form of mastocytosis diagnosed in adults, characterized by mast cell infiltration of one or more extracutaneous organs (with or without skin involvement). […] The management of patients with mastocytosis requires a multidisciplinary team approach (involving dermatologists, hematologists, gastroenterologists, pathologists, and allergists/immunologists) preferably in specialized medical centers with expertise in the treatment of patients with mast cell disorders. […] These NCCN Guidelines provide recommendations for the diagnosis and management of patients with SM. Management of CM is not included in these guidelines. Referral to centers with expertise in CM is strongly recommended.

• #8 Systemic Mastocytosis | Huntsman Cancer Institute | University of Utah Health

  https://healthcare.utah.edu/huntsmancancerinstitute/hematologic-cancers/systemic-mastocytosis

  Systemic mastocytosis is a rare disorder that happens when too many mast cells build up in parts of your body. […] Systemic mastocytosis originates in the bone marrow, and can happen in the skin, liver, spleen, stomach, small intestine, or colon. […] Your care team may include these specialists: Hematologists (Blood specialist doctors), pain and palliative care doctors, dermatologists, and doctors from other specialties, as needed. […] Our care teams may also include advanced care nurses, and physician assistants. Everyone on your care team talks with each other to plan and give treatment. They can talk to you about any questions or concerns you have about systemic mastocytosis.

• #9 Mastocytosis | Cancer Support Community

  https://www.cancersupportcommunity.org/mastocytosis

  Systemic mastocytosis (SM) occurs when too many mast cells grow and build up in the body. These cells may begin to grow in only one area of the body, like in the bone marrow, or in multiple tissues or organs. This form of mastocytosis is more common in adults. Symptoms of SM are typically treated with drugs like antihistamines. Antihistamines block the mediators found in the mast cells from being released. […] To develop the best treatment plan for your diagnosis, you may have a team of healthcare professionals from different backgrounds working together. You may see a dermatologist, a gastroenterologist, a hematologist, and an allergist or an immunologist. Mastocytosis is often misdiagnosed because it is rare. It is important to find a doctor who has experience diagnosing and treating mastocytosis.

• #10 Rare diseases | Systemic Mastocytosis

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

  Istituto Gentili is committed to the treatment of systemic mastocytosis, in order to improve the quality of life of Patients suffering from this rare and invalidating disease. […] The treatment of systemic mastocytosis is tailored to the individual Patient based on: severity of the symptoms, manifestations of the disease, clinical evolution and prognosis. […] As there is no pharmacological treatment that is able to cure mastocytosis, the therapeutic options available have three aims: reduce the symptoms, by controlling the secretion and effects of mast cell mediators, reduce the extent of mast cell infiltration with cytoreduction therapies, treat the complications, such as organ dysfunction, caused by mast cell infiltration. […] Given the complexity of the disease, the treatment and management of Patients with mastocytosis requires the involvement of medical specialties.

• #11 Systemic Mastocytosis, Version 2.2019, NCCN Clinical Practice Guidelines in Oncology in: Journal of the National Comprehensive Cancer Network Volume 16 Issue 12 (2018)

  https://jnccn.org/view/journals/jnccn/16/12/article-p1500.xml

  Referral to specialized centers with expertise in the management of mastocytosis is strongly recommended. […] Patients should be counseled about the signs and symptoms of mast cell activation and the importance of avoiding known triggers of mast cell activation. Anaphylactic reactions are significantly more frequent in patients with ISM and should be managed with the use of epinephrine injection. All patients should carry 2 auto injectors of epinephrine to manage anaphylaxis. […] Anti-mediator drug therapy for mast cell activation symptoms (as described in next section) is recommended for all patients with SM. […] Cytoreductive therapy (discussed in next section) is recommended for patients with advanced SM (ASM, SM-AHN, and MCL) because of the frequent presence of organ damage and shortened survival of this patient population.

• #12 Advanced Systemic Mastocytosis: Symptoms, Causes, Diagnosis, and Treatment

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

  Finding a healthcare team that is experienced in treating advanced systemic mastocytosis is essential to managing it effectively, says Gotlib. You can ask your healthcare provider to refer you to a mastocytosis center of excellence, which can provide access to the latest treatments and possibly clinical trials. […] The most meaningful lifestyle change for advanced systemic mastocytosis is to avoid the triggers that can lead to inflammation and organ damage. […] Although current guidelines don’t suggest any special diet for people with advanced systemic mastocytosis, some experts recommended a low-histamine diet. […] The prognosis for individuals with advanced systemic mastocytosis depends on several factors, including the extent of mast cell accumulation, the organs involved, and the person’s overall health. […] Support is available. People can benefit from connecting with others who understand their experiences. Support groups, both in-person and online, provide a sense of community and can be a valuable resource for coping strategies, emotional support, and information about new treatments.

• #13 Duke Blood Cancer Center Named Mast Cell Diseases Center of Excellence | Duke Health

  https://www.dukehealth.org/blog/duke-blood-cancer-center-named-mast-cell-diseases-center-of-excellence

  The Duke Blood Cancer Center has been recognized as a Center of Excellence by the American Initiative in Mast Cell Diseases Network. This designation acknowledges the centers expertise in caring for people with systemic mastocytosis and related mast cell disorders. […] The Duke Blood Cancer Center is one of a handful of centers in the Southeast with the experience and capability to treat mast cell diseases. […] Having experts in multiple specialties — including hematology, allergy and immunology, dermatology, and oncology — is essential for helping people manage these symptoms, treating their disease, and improving their quality of life. […] In addition to patient care, Dr. Rein and her colleagues are actively involved in research on new treatments for systemic mastocytosis and other mast cell diseases. […] While there is currently no cure for systemic mastocytosis and related mast cell disorders, receiving comprehensive care at a Mast Cell Diseases Center of Excellence can help people with these conditions live a full and fulfilling life.

• #14 Systemic Mastocytosis Treatment & Management: Medical Care, Surgical Care, Consultations

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

  Therapy for systemic mastocytosis (systemic mast cell disease) is primarily symptomatic; no therapy is curative. Treatment modalities include the management of (1) anaphylaxis and related symptoms, (2) pruritus and flushing, and (3) intestinal malabsorption. The principles of treatment include control of symptoms with measures to decrease mast cell activation. […] Antihistamines (H1 and H2 receptor blockers) are part of initial pharmacologic treatment in systemic mastocytosis. […] Cromolyn sodium has demonstrated multiple benefits in systemic mastocytosis. It has proved useful for relieving abdominal pain and diarrhea; pruritus, whealing, and flushing; and impaired cognition. […] Corticosteroids have been used to control malabsorption, ascites, abdominal pain refractory to cromolyn, bone pain, and diffuse cutaneous disease refractory to topical therapy, and to prevent anaphylaxis.

• #15 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. […] The objective of treatment is to control the effects of mast cell released mediators by avoidance of triggers, as well as the use of various medications. […] Most treatment is supportive, but there have been recent successes with targeted therapies. […] Antihistamines, including both H1 and H2 antihistamines, are commonly used to block the effects of mast cell histamine. […] Epinephrine may be required to treat episodes of anaphylaxis or low blood pressure. […] It is suggested that mastocytosis patients should carry two epinephrine injectors due to the increased mast cell burden and increased mediator release.

• #16 What are treatments for Systemic Mastocytosis? – HealthTree for Systemic Mastocytosis

  https://healthtree.org/mastocytosis/community/what-are-treatments-for-systemic-mastocytosis

  Antihistamines, such as cetirizine and loratadine, help manage skin symptoms like flushing and itching, as well as gastrointestinal issues like stomach pain, heartburn, cramping, and diarrhea. […] Mast cell stabilizers, such as cromolyn sodium, prevent mast cells from releasing harmful substances, helping with allergy-like symptoms like rash, itching, and eye inflammation. […] Injectable epinephrine is sometimes needed if patients experience a full-body allergic reaction called anaphylaxis. […] Monoclonal antibodies such as omalizumab (anti-IgE) can reduce the frequency of anaphylaxis. […] Steroids such as prednisone reduce inflammation and have been used to control gastrointestinal issues like malabsorption, fluid build-up in the abdomen, stomach pain, bone pain, and skin disease that doesn’t respond to topical treatment. Steroids can also be used to prevent anaphylaxis.

• #17 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. […] Pharmacologic treatment of mastocytosis involves stabilizing mast cell membranes to decrease the severity of the attacks while blocking the action of inflammatory mediators. […] 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. […] Systemic steroids may be necessary to control the severe skin disease, malabsorption or ascites of systemic mastocytosis.

• #18 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. […] Pharmacologic treatment of mastocytosis involves stabilizing mast cell membranes to decrease the severity of the attacks while blocking the action of inflammatory mediators. […] 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. […] Systemic steroids may be necessary to control the severe skin disease, malabsorption or ascites of systemic mastocytosis.

• #19 Mastocytosis (Mast Cell Disorder): Symptoms and Treatment

  https://patient.info/doctor/mastocytosis-and-mast-cell-disorders

  Mastocytosis treatment and management is concerned mainly with symptom control, as there is currently no cure. Systemic mastocytosis is usually managed by haematologists. […] Acute anaphylaxis is treated with intramuscular adrenaline (epinephrine), antihistamines (H1 and H2 receptor blockers), fluids and pressor agents. […] For pruritus, weals and flushing – H1 and H2 receptor antagonists such as chlorphenamine, ketotifen and cimetidine. […] There is some evidence that long-term use of antihistamines can affect cognition, so these drugs should be titrated to the lowest effective dose and used for the shortest possible time. […] Local corticosteroids for skin lesions. Intralesional steroid injection is sometimes used. […] Systemic corticosteroids may be helpful for malabsorption, ascites and bone pain, to prevent anaphylaxis and for severe skin disease. […] Guidelines for management in children are available.

• #20 Systemic Mastocytosis Treatment & Management: Medical Care, Surgical Care, Consultations

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

  Therapy for systemic mastocytosis (systemic mast cell disease) is primarily symptomatic; no therapy is curative. Treatment modalities include the management of (1) anaphylaxis and related symptoms, (2) pruritus and flushing, and (3) intestinal malabsorption. The principles of treatment include control of symptoms with measures to decrease mast cell activation. […] Antihistamines (H1 and H2 receptor blockers) are part of initial pharmacologic treatment in systemic mastocytosis. […] Cromolyn sodium has demonstrated multiple benefits in systemic mastocytosis. It has proved useful for relieving abdominal pain and diarrhea; pruritus, whealing, and flushing; and impaired cognition. […] Corticosteroids have been used to control malabsorption, ascites, abdominal pain refractory to cromolyn, bone pain, and diffuse cutaneous disease refractory to topical therapy, and to prevent anaphylaxis.

• #21 What are treatments for Systemic Mastocytosis? – HealthTree for Systemic Mastocytosis

  https://healthtree.org/mastocytosis/community/what-are-treatments-for-systemic-mastocytosis

  Antihistamines, such as cetirizine and loratadine, help manage skin symptoms like flushing and itching, as well as gastrointestinal issues like stomach pain, heartburn, cramping, and diarrhea. […] Mast cell stabilizers, such as cromolyn sodium, prevent mast cells from releasing harmful substances, helping with allergy-like symptoms like rash, itching, and eye inflammation. […] Injectable epinephrine is sometimes needed if patients experience a full-body allergic reaction called anaphylaxis. […] Monoclonal antibodies such as omalizumab (anti-IgE) can reduce the frequency of anaphylaxis. […] Steroids such as prednisone reduce inflammation and have been used to control gastrointestinal issues like malabsorption, fluid build-up in the abdomen, stomach pain, bone pain, and skin disease that doesn’t respond to topical treatment. Steroids can also be used to prevent anaphylaxis.

• #22 Systemic Mastocytosis Treatment & Management: Medical Care, Surgical Care, Consultations

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

  Therapy for systemic mastocytosis (systemic mast cell disease) is primarily symptomatic; no therapy is curative. Treatment modalities include the management of (1) anaphylaxis and related symptoms, (2) pruritus and flushing, and (3) intestinal malabsorption. The principles of treatment include control of symptoms with measures to decrease mast cell activation. […] Antihistamines (H1 and H2 receptor blockers) are part of initial pharmacologic treatment in systemic mastocytosis. […] Cromolyn sodium has demonstrated multiple benefits in systemic mastocytosis. It has proved useful for relieving abdominal pain and diarrhea; pruritus, whealing, and flushing; and impaired cognition. […] Corticosteroids have been used to control malabsorption, ascites, abdominal pain refractory to cromolyn, bone pain, and diffuse cutaneous disease refractory to topical therapy, and to prevent anaphylaxis.

• #23 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. […] Pharmacologic treatment of mastocytosis involves stabilizing mast cell membranes to decrease the severity of the attacks while blocking the action of inflammatory mediators. […] 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. […] Systemic steroids may be necessary to control the severe skin disease, malabsorption or ascites of systemic mastocytosis.

• #24 What are treatments for Systemic Mastocytosis? – HealthTree for Systemic Mastocytosis

  https://healthtree.org/mastocytosis/community/what-are-treatments-for-systemic-mastocytosis

  Antihistamines, such as cetirizine and loratadine, help manage skin symptoms like flushing and itching, as well as gastrointestinal issues like stomach pain, heartburn, cramping, and diarrhea. […] Mast cell stabilizers, such as cromolyn sodium, prevent mast cells from releasing harmful substances, helping with allergy-like symptoms like rash, itching, and eye inflammation. […] Injectable epinephrine is sometimes needed if patients experience a full-body allergic reaction called anaphylaxis. […] Monoclonal antibodies such as omalizumab (anti-IgE) can reduce the frequency of anaphylaxis. […] Steroids such as prednisone reduce inflammation and have been used to control gastrointestinal issues like malabsorption, fluid build-up in the abdomen, stomach pain, bone pain, and skin disease that doesn’t respond to topical treatment. Steroids can also be used to prevent anaphylaxis.

• #25 Mastocytosis (Mast Cell Disorder): Symptoms and Treatment

  https://patient.info/doctor/mastocytosis-and-mast-cell-disorders

  Mastocytosis treatment and management is concerned mainly with symptom control, as there is currently no cure. Systemic mastocytosis is usually managed by haematologists. […] Acute anaphylaxis is treated with intramuscular adrenaline (epinephrine), antihistamines (H1 and H2 receptor blockers), fluids and pressor agents. […] For pruritus, weals and flushing – H1 and H2 receptor antagonists such as chlorphenamine, ketotifen and cimetidine. […] There is some evidence that long-term use of antihistamines can affect cognition, so these drugs should be titrated to the lowest effective dose and used for the shortest possible time. […] Local corticosteroids for skin lesions. Intralesional steroid injection is sometimes used. […] Systemic corticosteroids may be helpful for malabsorption, ascites and bone pain, to prevent anaphylaxis and for severe skin disease. […] Guidelines for management in children are available.

• #26 Systemic Mastocytosis Treatment & Management: Medical Care, Surgical Care, Consultations

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

  Antiresorptive treatment with bisphosphonates is a rational treatment for osteoporosis in patients with systemic mastocytosis. […] Medical therapy for systemic mastocytosis is generally considered as initial treatment for patients with advanced systemic mastocytosis (aggressive systemic mastocytosis, systemic mastocytosis with an associated hematological neoplasm, mast cell leukemia), as bridging treatment to undergo allogenic hematopoietic cell transplantation, or in selected patients with indolent systemic mastocytosis or smoldering systemic mastocytosis who suffer recurrent anaphylaxis despite treatment with all other options. […] Patients thought to have severe systemic mastocytosis that requires chemotherapy may need consultation with hematologists, dermatologists, and immunologists. […] Patients with systemic mastocytosis should exercise great care in avoiding stings when engaging in outdoor activities. […] Patients should carry an epinephrine autoinjector at all times and should be taught to use the device in case of emergency.

• #27 Systemic Mastocytosis, Version 2.2019, NCCN Clinical Practice Guidelines in Oncology in: Journal of the National Comprehensive Cancer Network Volume 16 Issue 12 (2018)

  https://jnccn.org/view/journals/jnccn/16/12/article-p1500.xml

  Enrollment in well-designed clinical trials investigating novel therapeutic strategies (eg, selective KIT D816 inhibitors) is encouraged to enable further advances. […] The use of bisphosphonates (with continued use of antihistamines) is recommended to resolve bone pain and improve vertebral bone mineral density (BMD). […] Denosumab, an anti-RANKL monoclonal antibody, has also been associated with significant increases in BMD at lumbar and femoral sites, decreases in bone turnover markers in serum. […] Allogeneic hematopoietic cell transplant (HCT) has been evaluated in patients with advanced SM, and the outcomes are significantly affected by the subtype of SM and the type of conditioning regimen used. […] The role of allogeneic HCT needs to be determined in a prospective trial. However, given the rarity of SM, no larger prospective trials of HCT have been initiated to confirm the role of allogeneic HCT.

• #28 Systemic Mastocytosis, Version 2.2019, NCCN Clinical Practice Guidelines in Oncology in: Journal of the National Comprehensive Cancer Network Volume 16 Issue 12 (2018)

  https://jnccn.org/view/journals/jnccn/16/12/article-p1500.xml

  Enrollment in well-designed clinical trials investigating novel therapeutic strategies (eg, selective KIT D816 inhibitors) is encouraged to enable further advances. […] The use of bisphosphonates (with continued use of antihistamines) is recommended to resolve bone pain and improve vertebral bone mineral density (BMD). […] Denosumab, an anti-RANKL monoclonal antibody, has also been associated with significant increases in BMD at lumbar and femoral sites, decreases in bone turnover markers in serum. […] Allogeneic hematopoietic cell transplant (HCT) has been evaluated in patients with advanced SM, and the outcomes are significantly affected by the subtype of SM and the type of conditioning regimen used. […] The role of allogeneic HCT needs to be determined in a prospective trial. However, given the rarity of SM, no larger prospective trials of HCT have been initiated to confirm the role of allogeneic HCT.

• #29 Systemic Mastocytosis, Version 2.2019, NCCN Clinical Practice Guidelines in Oncology in: Journal of the National Comprehensive Cancer Network Volume 16 Issue 12 (2018)

  https://jnccn.org/view/journals/jnccn/16/12/article-p1500.xml

  Referral to specialized centers with expertise in the management of mastocytosis is strongly recommended. […] Patients should be counseled about the signs and symptoms of mast cell activation and the importance of avoiding known triggers of mast cell activation. Anaphylactic reactions are significantly more frequent in patients with ISM and should be managed with the use of epinephrine injection. All patients should carry 2 auto injectors of epinephrine to manage anaphylaxis. […] Anti-mediator drug therapy for mast cell activation symptoms (as described in next section) is recommended for all patients with SM. […] Cytoreductive therapy (discussed in next section) is recommended for patients with advanced SM (ASM, SM-AHN, and MCL) because of the frequent presence of organ damage and shortened survival of this patient population.

• #30 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. […] General anesthesia may be problematic in patients with systemic mastocytosis and perioperative modalities of these patients need to be considered. […] The treatment algorithm for systemic mastocytosis is complex, and the condition is primarily managed by a hematologist. […] Patients with aggressive systemic mastocytosis have disease-related organ dysfunction; interferon-alfa (with or without corticosteroids) can control dermatological, hematological, GI, skeletal, and mediator-release symptoms, but may be poorly tolerated. […] Emergency resuscitation or hospitalization may be required for severe syncope or hypotensive shock resulting from the sudden severe degranulation of many mast cells.

• #31 Leukemia and Other Blood Cancers: Systemic Mastocytosis | Memorial Sloan Kettering Cancer Center

  https://www.mskcc.org/cancer-care/types/leukemias/types/systemic-mastocytosis

  Mastocytosis happens when too many mast cells build up in the body. Mast cells are a type of white blood cell that play an important role in helping the immune system defend the body from disease. They also respond to allergic reactions, help heal wounds, and defend against infections. […] In systemic mastocytosis, however, there are too many mast cells throughout the body, including in the gastrointestinal tract and the bone marrow. When the disease is in the intestinal tract, it can lead to nausea, vomiting, diarrhea, and abdominal pain. […] Our doctors typically treat the illness with antihistamines, drugs that are used for people with allergies. For more severe cases, we may recommend steroid or chemotherapy treatment. A targeted drug called midostaurin (Rydapt) is approved to treat more-aggressive cases of systemic mastocytosis. Finally, our scientists are researching new drugs for people with systemic mastocytosis that carries certain mutations.

• #32 Quest to find better treatments for rare blood cancer leads to new therapies | News Center

  https://med.stanford.edu/news/all-news/2022/03/systemic-mastocytosis-therapies.html

  „Overall, her quality of life was quite poor,” Gotlib said. […] Although a medication called imatinib, which is sold under the brand name Gleevec, has been approved for use in advanced SM, it is ineffective against the D816V mutation (although it may be useful in the rare SM patients who have normal KIT or other mutations in the gene). […] Gotlib suggested Worthen enroll in a phase-1 clinical trial he was leading of a drug called avapritinib, which has a mechanism of action similar to midostaurin’s but is less likely to cause severe side effects because it targets only the KIT protein. […] „Avapritinib is considerably more selective than midostaurin,” Gotlib said. „It was specifically designed to inhibit the KIT D816V mutation with very few other targets. Midostaurin was a big step forward in the treatment of advanced SM, but it hits several other targets, and avapritinib is more potent.”

• #33 Living With Systemic Mastocytosis: Self-Care Tips – The Doctor’s Channel

  https://www.thedoctorschannel.com/view/living-with-systemic-mastocytosis-self-care-tips/

  Systemic mastocytosis, a rare blood disorder, affects the entire body. There are many challenges associated with living with this systemic mastocytosis that are centered around reducing the frequency and severity of symptoms. Optimizing mental health, engaging in low-impact exercises, improving sleep hygiene, and limiting food triggers can contribute to successful symptom management. […] Systemic mastocytosis with associated hematologic neoplasm (SM-AHN) presents unique diagnostic and treatment challenges. In this Medscape-certified activity, experts discuss the complex patient journey, from delayed diagnosis to individualized […] Avapritinib, branded as Ayvakit, became the first FDA-approved treatment for indolent systemic mastocytosis (ISM) in 2023, marking a major advancement for patients. ISM, driven by the KIT D816V

• #34 Systemic Mastocytosis: Symptoms, Causes & Treatment

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

  Healthcare providers cant cure the condition. But treatment can ease your symptoms. […] Healthcare providers treat systemic mastocytosis by managing symptoms and complications. For example, if you have excess stomach acid, they may prescribe H2 blockers (antacids). If the condition causes anemia, theyll treat the anemia. […] Systemic mastocytosis treatments may include: Antihistamines to relieve itching, flushing and other skin reactions; Corticosteroids to reduce inflammation; Bisphosphonates to help with bone weakness; Targeted therapy, which focuses on the protein in abnormal mast cells; Chemotherapy, if systemic mastocytosis becomes cancerous; Splenectomy to remove an enlarged spleen; Bone marrow transplant to treat aggressive or advanced systemic mastocytosis. […] If you have systemic mastocytosis, you should always carry an EpiPen. Epinephrine treats anaphylactic shock, a severe, life-threatening allergic reaction.

• #35 Quest to find better treatments for rare blood cancer leads to new therapies | News Center

  https://med.stanford.edu/news/all-news/2022/03/systemic-mastocytosis-therapies.html

  Of the 53 patients with advanced SM evaluated in the EXPLORER trial, 75% experienced at least some benefit from the drug and 36% of patients experienced a complete remission. The drug is not without side effects, which may include facial swelling, diarrhea, nausea and fatigue. There is also a risk of intracranial bleeding in people with low platelet counts. […] „We now have two drugs approved for the front-line treatment of advanced SM,” Gotlib said. „This is a really beautiful example of the effectiveness of targeted therapy for a rare disease, and I’m delighted to see how it has improved the lives of these patients.” […] The treatment allowed Worthen to get her life back, including a return to horseback riding. She now relies on regular blood tests to ensure her disease has not returned. „Slowly I got stronger, and I’m now feeling back to where I was before I became ill,” she said. „I feel very, very lucky and thankful.”

• #36 What are treatments for Systemic Mastocytosis? – HealthTree for Systemic Mastocytosis

  https://healthtree.org/mastocytosis/community/what-are-treatments-for-systemic-mastocytosis

  Antihistamines, such as cetirizine and loratadine, help manage skin symptoms like flushing and itching, as well as gastrointestinal issues like stomach pain, heartburn, cramping, and diarrhea. […] Mast cell stabilizers, such as cromolyn sodium, prevent mast cells from releasing harmful substances, helping with allergy-like symptoms like rash, itching, and eye inflammation. […] Injectable epinephrine is sometimes needed if patients experience a full-body allergic reaction called anaphylaxis. […] Monoclonal antibodies such as omalizumab (anti-IgE) can reduce the frequency of anaphylaxis. […] Steroids such as prednisone reduce inflammation and have been used to control gastrointestinal issues like malabsorption, fluid build-up in the abdomen, stomach pain, bone pain, and skin disease that doesn’t respond to topical treatment. Steroids can also be used to prevent anaphylaxis.

• #37 Anaphylaxis threatens patients with systemic mastocytosis: A new study separates patients at high risk of anaphylaxis from those at low risk, a help in care – TMS – The Mast Cell Disease Society, IncAccessibilityIncrease TextDecrease TextGrayscaleHigh Con

  https://tmsforacure.org/anaphylaxis-threatens-patients-with-systemic-mastocytosis-a-new-study-separates-patients-at-high-risk-of-anaphylaxis-from-those-at-low-risk-a-help-in-care/

  Anaphylaxis threatens patients with systemic mastocytosis: A new study separates patients at high risk of anaphylaxis from those at low risk, a help in care. Mastocytosis is characterized by an accumulation of clonal mast cells in the skin and/or internal organs. Systemic mastocytosis also involves at least one organ outside the skin, most often the bone marrow. […] In conclusion, the authors have developed a potential preliminary tool to separate patients with SM at high risk of anaphylaxis from those at low risk. They hope this tool may help improve the care of patients and may aid physicians to more accurately assess anaphylaxis risk, resulting in improved quality of life for patients. […] We strongly urge all patients with all forms of mast cell disease to carry two doses of auto-injectable epinephrine at all times, and to be trained, along with their caregivers, on how to administer it.

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

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

  Systemic mastocytosis care at Mayo Clinic […] Our caring team of Mayo Clinic experts can help you with your systemic mastocytosis-related health concerns Start Here […] 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. […] A health care professional can teach you how to give yourself an epinephrine injection in the event that you have a severe allergic response when your mast cells are triggered. […] 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.

• #39 Mastocytosis: Types, Symptoms & Treatment

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

  Most people can manage mastocytosis symptoms with medication and by avoiding activities and environments that trigger the condition. […] Healthcare providers cant cure mastocytosis, but they can treat its symptoms and the complications the condition causes. […] Treatment varies based on mastocytosis type and your symptoms. In general, providers use medications and other treatments to ease symptoms. […] Providers may use a variety of treatments for mastocytosis symptoms. Most treatments have side effects, some more serious than others. If you have mastocytosis, your provider will explain what you can expect, including treatment side effects. […] 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.

• #40 Anaphylaxis threatens patients with systemic mastocytosis: A new study separates patients at high risk of anaphylaxis from those at low risk, a help in care – TMS – The Mast Cell Disease Society, IncAccessibilityIncrease TextDecrease TextGrayscaleHigh Con

  https://tmsforacure.org/anaphylaxis-threatens-patients-with-systemic-mastocytosis-a-new-study-separates-patients-at-high-risk-of-anaphylaxis-from-those-at-low-risk-a-help-in-care/

  Anaphylaxis threatens patients with systemic mastocytosis: A new study separates patients at high risk of anaphylaxis from those at low risk, a help in care. Mastocytosis is characterized by an accumulation of clonal mast cells in the skin and/or internal organs. Systemic mastocytosis also involves at least one organ outside the skin, most often the bone marrow. […] In conclusion, the authors have developed a potential preliminary tool to separate patients with SM at high risk of anaphylaxis from those at low risk. They hope this tool may help improve the care of patients and may aid physicians to more accurately assess anaphylaxis risk, resulting in improved quality of life for patients. […] We strongly urge all patients with all forms of mast cell disease to carry two doses of auto-injectable epinephrine at all times, and to be trained, along with their caregivers, on how to administer it.

• #41 Critical care management of systemic mastocytosis: when every wasp is a killer bee

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4453286/

  Since the critical care physician will most likely be involved in a life-threatening expression of systemic mastocytosis, recognition of this disease is of utmost importance in the critical care management of these patients. […] The mainstay of therapy is the avoidance of potential triggers of mast cell degranulation and, if unsuccessful, blocking the cascade of mast cell mediators. […] The critical care physician should be well aware of the special precautions which should be kept in mind throughout the management of a mastocytosis crisis to avoid massive mast cell degranulation. […] For all patients with mastocytosis, epinephrine should be readily available as it hinders mast cell degranulation and is the first drug of choice during haemodynamic failure in these patients. […] It is important to realise that, during further ICU treatment, patients with mastocytosis are inevitably exposed to triggers of mast cell degranulation, which may be followed by severe anaphylactic responses.

• #42 Mastocytosis (Mast Cell Disorder): Symptoms and Treatment

  https://patient.info/doctor/mastocytosis-and-mast-cell-disorders

  Mastocytosis treatment and management is concerned mainly with symptom control, as there is currently no cure. Systemic mastocytosis is usually managed by haematologists. […] Acute anaphylaxis is treated with intramuscular adrenaline (epinephrine), antihistamines (H1 and H2 receptor blockers), fluids and pressor agents. […] For pruritus, weals and flushing – H1 and H2 receptor antagonists such as chlorphenamine, ketotifen and cimetidine. […] There is some evidence that long-term use of antihistamines can affect cognition, so these drugs should be titrated to the lowest effective dose and used for the shortest possible time. […] Local corticosteroids for skin lesions. Intralesional steroid injection is sometimes used. […] Systemic corticosteroids may be helpful for malabsorption, ascites and bone pain, to prevent anaphylaxis and for severe skin disease. […] Guidelines for management in children are available.

• #43 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. […] General anesthesia may be problematic in patients with systemic mastocytosis and perioperative modalities of these patients need to be considered. […] The treatment algorithm for systemic mastocytosis is complex, and the condition is primarily managed by a hematologist. […] Patients with aggressive systemic mastocytosis have disease-related organ dysfunction; interferon-alfa (with or without corticosteroids) can control dermatological, hematological, GI, skeletal, and mediator-release symptoms, but may be poorly tolerated. […] Emergency resuscitation or hospitalization may be required for severe syncope or hypotensive shock resulting from the sudden severe degranulation of many mast cells.

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

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

  Systemic mastocytosis care at Mayo Clinic […] Our caring team of Mayo Clinic experts can help you with your systemic mastocytosis-related health concerns Start Here […] 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. […] A health care professional can teach you how to give yourself an epinephrine injection in the event that you have a severe allergic response when your mast cells are triggered. […] 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.

• #45 Critical care management of systemic mastocytosis: when every wasp is a killer bee

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4453286/

  Histamine-releasing drugs, such as opioids and neuromuscular blocking agents, should be avoided. […] When (surgical) intervention is planned during ICU admission, precautions must be taken. […] Knowledge of the pathophysiology of the disease is of paramount importance for adequate treatment and the prevention of secondary events of mast cell degranulation due to mechanical or pharmacological triggers. […] During treatment, physicians should avoid potential triggers of mast cell degranulation, consider giving pre-medication before interventions, and be aware of and prepared for the clinical manifestations caused by mast cell mediators. […] Avoiding medication and physical stimuli that trigger histamine release and blocking the cascade of actions of mast cells constitute the mainstay of therapy besides treatment of anaphylaxis.

• #46 Systemic Mastocytosis: Symptoms, Subtypes, Treatment

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

  Mast cell stabilizers: Medications like cromolyn sodium can prevent mast cells from releasing histamine and other chemicals, reducing symptoms and flare-ups […] Proton pump inhibitors (PPIs) and H2 blockers: These are used to reduce stomach acid production and help manage gastrointestinal symptoms […] Corticosteroids: In severe cases, corticosteroids may be prescribed to reduce inflammation and control symptoms. […] Systemic mastocytosis can worsen due to triggers that prompt mast cells to release histamine and other chemicals. While triggers can vary among individuals, common ones include: […] Exposure to extreme temperatures […] Intense exercise or physical activity […] Rubbing or scratching the skin […] Spicy foods, alcohol, and foods high in histamine or tyramine (such as aged cheeses and fermented products) […] Living with systemic mastocytosis can be challenging, but understanding the condition and connecting with supportive resources can make a significant difference.

• #47 Systemic Mastocytosis: 6 Healthy Habits

  https://www.healthline.com/health/allergies/systemic-mastocytosis-healthy-habits

  If you have systemic mastocytosis, you probably have a personalized treatment plan. You know how important it is to take medications as prescribed. […] But managing this condition takes more than medication. Living with mastocytosis means being on alert for potential triggers and taking steps to avoid them. […] Although triggers vary from person to person, some basic healthy habits may help as you navigate life with systemic mastocytosis. […] Stress is the most common trigger of mastocytosis. […] Foods are known triggers of anaphylaxis in people with mastocytosis. […] Two triggers that often go together are exercise and temperature changes. […] If you tend to have skin symptoms, such as rash, hives, or itching, give your skin a little extra care by keeping skin well moisturized.

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

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

  Therapy is conservative and aimed at symptom relief because the prognosis for most patients with cutaneous mastocytosis (CM) is excellent. None of the currently available therapeutic measures induces permanent involution of cutaneous or visceral lesions. Advise patients to avoid agents that precipitate mediator release, such as aspirin, nonsteroidal anti-inflammatory drugs, codeine, morphine, alcohol, thiamine, quinine, opiates, gallamine, decamethonium, procaine, radiographic dyes, dextran, polymyxin B, scopolamine, and D-tubocurarine. […] H1 and H2 antihistamines decrease pruritus, flushing, and GI symptoms. Oral disodium cromoglycate may ameliorate cutaneous symptoms, such as pruritus, whealing, and flushing, as well as systemic symptoms, such as diarrhea, abdominal pain, bone pain, and disorders of cognitive function.

• #49 Critical care management of systemic mastocytosis: when every wasp is a killer bee

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4453286/

  Histamine-releasing drugs, such as opioids and neuromuscular blocking agents, should be avoided. […] When (surgical) intervention is planned during ICU admission, precautions must be taken. […] Knowledge of the pathophysiology of the disease is of paramount importance for adequate treatment and the prevention of secondary events of mast cell degranulation due to mechanical or pharmacological triggers. […] During treatment, physicians should avoid potential triggers of mast cell degranulation, consider giving pre-medication before interventions, and be aware of and prepared for the clinical manifestations caused by mast cell mediators. […] Avoiding medication and physical stimuli that trigger histamine release and blocking the cascade of actions of mast cells constitute the mainstay of therapy besides treatment of anaphylaxis.

• #50 Systemic Mastocytosis Treatment & Management: Medical Care, Surgical Care, Consultations

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

  Antiresorptive treatment with bisphosphonates is a rational treatment for osteoporosis in patients with systemic mastocytosis. […] Medical therapy for systemic mastocytosis is generally considered as initial treatment for patients with advanced systemic mastocytosis (aggressive systemic mastocytosis, systemic mastocytosis with an associated hematological neoplasm, mast cell leukemia), as bridging treatment to undergo allogenic hematopoietic cell transplantation, or in selected patients with indolent systemic mastocytosis or smoldering systemic mastocytosis who suffer recurrent anaphylaxis despite treatment with all other options. […] Patients thought to have severe systemic mastocytosis that requires chemotherapy may need consultation with hematologists, dermatologists, and immunologists. […] Patients with systemic mastocytosis should exercise great care in avoiding stings when engaging in outdoor activities. […] Patients should carry an epinephrine autoinjector at all times and should be taught to use the device in case of emergency.

• #51 Systemic Mastocytosis: Symptoms, Subtypes, Treatment

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

  Mast cell stabilizers: Medications like cromolyn sodium can prevent mast cells from releasing histamine and other chemicals, reducing symptoms and flare-ups […] Proton pump inhibitors (PPIs) and H2 blockers: These are used to reduce stomach acid production and help manage gastrointestinal symptoms […] Corticosteroids: In severe cases, corticosteroids may be prescribed to reduce inflammation and control symptoms. […] Systemic mastocytosis can worsen due to triggers that prompt mast cells to release histamine and other chemicals. While triggers can vary among individuals, common ones include: […] Exposure to extreme temperatures […] Intense exercise or physical activity […] Rubbing or scratching the skin […] Spicy foods, alcohol, and foods high in histamine or tyramine (such as aged cheeses and fermented products) […] Living with systemic mastocytosis can be challenging, but understanding the condition and connecting with supportive resources can make a significant difference.

• #52 Systemic Mastocytosis: Symptoms, Causes & Treatment

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

  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. […] Systemic mastocytosis is a life-changing diagnosis. It means youll visit your healthcare provider often. Blood tests will become routine. Youll also need to identify triggers and try to avoid them. Living with this condition is a big adjustment. Lean on your healthcare provider. Theyre here to help. They can point you to resources and support groups that can empower you to take control of your health.

• #53 Systemic Mastocytosis Treatment & Management: Medical Care, Surgical Care, Consultations

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

  Antiresorptive treatment with bisphosphonates is a rational treatment for osteoporosis in patients with systemic mastocytosis. […] Medical therapy for systemic mastocytosis is generally considered as initial treatment for patients with advanced systemic mastocytosis (aggressive systemic mastocytosis, systemic mastocytosis with an associated hematological neoplasm, mast cell leukemia), as bridging treatment to undergo allogenic hematopoietic cell transplantation, or in selected patients with indolent systemic mastocytosis or smoldering systemic mastocytosis who suffer recurrent anaphylaxis despite treatment with all other options. […] Patients thought to have severe systemic mastocytosis that requires chemotherapy may need consultation with hematologists, dermatologists, and immunologists. […] Patients with systemic mastocytosis should exercise great care in avoiding stings when engaging in outdoor activities. […] Patients should carry an epinephrine autoinjector at all times and should be taught to use the device in case of emergency.

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

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

  Systemic mastocytosis is a disease where too many abnormal mast cells (a type of white blood cell) build up in your skin and other organs. […] There’s no cure, but treatments can help you manage the condition. […] Consider reaching out to family and friends for support as you manage this disease. They can help you deal with the emotions and stress that come with the challenges of a long-term condition like 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. […] 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. […] If you find that the challenges of managing your disease are making you depressed or anxious, talk to your doctor. They can put you in touch with mental health professionals who are experts in treating these problems.

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

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

  Systemic mastocytosis can be a complex disease. Understanding your condition is critical to ongoing care and prevention of complications. […] Follow your doctor’s recommended care and ongoing monitoring. […] Caring for a lifelong disorder such as systemic mastocytosis can be stressful and exhausting. Consider these strategies: […] You’ll need to make important decisions about care. Medical centers with specialty teams can offer you information about systemic mastocytosis, as well as advice and support, and can help you manage care. […] Ask for or accept help from family and friends when needed. Take time for your interests and activities. Counseling with a mental health professional may help with adjustment and coping.

• #56 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. […] General anesthesia may be problematic in patients with systemic mastocytosis and perioperative modalities of these patients need to be considered. […] The treatment algorithm for systemic mastocytosis is complex, and the condition is primarily managed by a hematologist. […] Patients with aggressive systemic mastocytosis have disease-related organ dysfunction; interferon-alfa (with or without corticosteroids) can control dermatological, hematological, GI, skeletal, and mediator-release symptoms, but may be poorly tolerated. […] Emergency resuscitation or hospitalization may be required for severe syncope or hypotensive shock resulting from the sudden severe degranulation of many mast cells.

• #57 Critical care management of systemic mastocytosis: when every wasp is a killer bee | Critical Care | Full Text

  https://ccforum.biomedcentral.com/articles/10.1186/s13054-015-0956-z

  When a patient with known mastocytosis is admitted to the ICU, it may be helpful to make a risk profile to estimate the chance of developing anaphylactoid reactions. […] Histamine-releasing drugs, such as opioids and neuromuscular blocking agents, should be avoided. […] When (surgical) intervention is planned during ICU admission, precautions must be taken. […] Pre-medication may include benzodiazepines, H1- and H2-receptor antagonists to block the cascade of mast cell mediators, and glucocorticoids as anti-inflammatory and mast cell stabilizers. […] Knowledge of the pathophysiology of the disease is of paramount importance for adequate treatment and the prevention of secondary events of mast cell degranulation due to mechanical or pharmacological triggers. […] During treatment, physicians should avoid potential triggers of mast cell degranulation, consider giving pre-medication before interventions, and be aware of and prepared for the clinical manifestations caused by mast cell mediators.

• #58 Critical care management of systemic mastocytosis: when every wasp is a killer bee | Critical Care | Full Text

  https://ccforum.biomedcentral.com/articles/10.1186/s13054-015-0956-z

  When a patient with known mastocytosis is admitted to the ICU, it may be helpful to make a risk profile to estimate the chance of developing anaphylactoid reactions. […] Histamine-releasing drugs, such as opioids and neuromuscular blocking agents, should be avoided. […] When (surgical) intervention is planned during ICU admission, precautions must be taken. […] Pre-medication may include benzodiazepines, H1- and H2-receptor antagonists to block the cascade of mast cell mediators, and glucocorticoids as anti-inflammatory and mast cell stabilizers. […] Knowledge of the pathophysiology of the disease is of paramount importance for adequate treatment and the prevention of secondary events of mast cell degranulation due to mechanical or pharmacological triggers. […] During treatment, physicians should avoid potential triggers of mast cell degranulation, consider giving pre-medication before interventions, and be aware of and prepared for the clinical manifestations caused by mast cell mediators.

• #59 Critical care management of systemic mastocytosis: when every wasp is a killer bee

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4453286/

  Since the critical care physician will most likely be involved in a life-threatening expression of systemic mastocytosis, recognition of this disease is of utmost importance in the critical care management of these patients. […] The mainstay of therapy is the avoidance of potential triggers of mast cell degranulation and, if unsuccessful, blocking the cascade of mast cell mediators. […] The critical care physician should be well aware of the special precautions which should be kept in mind throughout the management of a mastocytosis crisis to avoid massive mast cell degranulation. […] For all patients with mastocytosis, epinephrine should be readily available as it hinders mast cell degranulation and is the first drug of choice during haemodynamic failure in these patients. […] It is important to realise that, during further ICU treatment, patients with mastocytosis are inevitably exposed to triggers of mast cell degranulation, which may be followed by severe anaphylactic responses.

• #60 Systemic Mastocytosis, Version 2.2019, NCCN Clinical Practice Guidelines in Oncology in: Journal of the National Comprehensive Cancer Network Volume 16 Issue 12 (2018)

  https://jnccn.org/view/journals/jnccn/16/12/article-p1500.xml

  Enrollment in well-designed clinical trials investigating novel therapeutic strategies (eg, selective KIT D816 inhibitors) is encouraged to enable further advances. […] The use of bisphosphonates (with continued use of antihistamines) is recommended to resolve bone pain and improve vertebral bone mineral density (BMD). […] Denosumab, an anti-RANKL monoclonal antibody, has also been associated with significant increases in BMD at lumbar and femoral sites, decreases in bone turnover markers in serum. […] Allogeneic hematopoietic cell transplant (HCT) has been evaluated in patients with advanced SM, and the outcomes are significantly affected by the subtype of SM and the type of conditioning regimen used. […] The role of allogeneic HCT needs to be determined in a prospective trial. However, given the rarity of SM, no larger prospective trials of HCT have been initiated to confirm the role of allogeneic HCT.

• #61 What are treatments for Systemic Mastocytosis? – HealthTree for Systemic Mastocytosis

  https://healthtree.org/mastocytosis/community/what-are-treatments-for-systemic-mastocytosis

  For patients with life-threatening systemic mastocytosis, allogeneic stem cell transplantation may be considered. This involves replacing the patient’s diseased bone marrow (which produces mast cells) with healthy bone marrow from a donor. This can potentially cure the disease, but it carries significant risks, including infection, graft-versus-host disease, and even death. Therefore, it is usually reserved for the most severe cases.

• #62 Systemic Mastocytosis: Symptoms, Causes & Treatment

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

  Healthcare providers cant cure the condition. But treatment can ease your symptoms. […] Healthcare providers treat systemic mastocytosis by managing symptoms and complications. For example, if you have excess stomach acid, they may prescribe H2 blockers (antacids). If the condition causes anemia, theyll treat the anemia. […] Systemic mastocytosis treatments may include: Antihistamines to relieve itching, flushing and other skin reactions; Corticosteroids to reduce inflammation; Bisphosphonates to help with bone weakness; Targeted therapy, which focuses on the protein in abnormal mast cells; Chemotherapy, if systemic mastocytosis becomes cancerous; Splenectomy to remove an enlarged spleen; Bone marrow transplant to treat aggressive or advanced systemic mastocytosis. […] If you have systemic mastocytosis, you should always carry an EpiPen. Epinephrine treats anaphylactic shock, a severe, life-threatening allergic reaction.

• #63 Systemic Mastocytosis: Symptoms, Causes & Treatment

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

  Healthcare providers cant cure the condition. But treatment can ease your symptoms. […] Healthcare providers treat systemic mastocytosis by managing symptoms and complications. For example, if you have excess stomach acid, they may prescribe H2 blockers (antacids). If the condition causes anemia, theyll treat the anemia. […] Systemic mastocytosis treatments may include: Antihistamines to relieve itching, flushing and other skin reactions; Corticosteroids to reduce inflammation; Bisphosphonates to help with bone weakness; Targeted therapy, which focuses on the protein in abnormal mast cells; Chemotherapy, if systemic mastocytosis becomes cancerous; Splenectomy to remove an enlarged spleen; Bone marrow transplant to treat aggressive or advanced systemic mastocytosis. […] If you have systemic mastocytosis, you should always carry an EpiPen. Epinephrine treats anaphylactic shock, a severe, life-threatening allergic reaction.

• #64 Mastocytosis: Types, Symptoms & Treatment

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

  Mastocytosis is an incurable disease. Different types of mastocytosis affect people in different ways. If youre living with mastocytosis, here are some general suggestions that may help: […] You’ll have regular checkups with your healthcare provider so they can monitor your symptoms and, if needed, adjust your treatment. Contact your provider any time you notice new symptoms or your symptoms are worse. […] Mastocytosis is a rare condition that happens when your body makes abnormal mast cells that multiply uncontrollably, affecting your skin and organs. Most people with mastocytosis manage its symptoms by knowing what triggers mastocytosis and by taking medication. People with this condition should also have self-injecting syringes of epinephrine at hand to treat severe allergic reactions. […] Cleveland Clinic can help you manage that with personalized treatment.

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

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

  Systemic mastocytosis care at Mayo Clinic […] Our caring team of Mayo Clinic experts can help you with your systemic mastocytosis-related health concerns Start Here […] 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. […] A health care professional can teach you how to give yourself an epinephrine injection in the event that you have a severe allergic response when your mast cells are triggered. […] 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.

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

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

  Systemic mastocytosis care at Mayo Clinic […] Our caring team of Mayo Clinic experts can help you with your systemic mastocytosis-related health concerns Start Here […] 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. […] A health care professional can teach you how to give yourself an epinephrine injection in the event that you have a severe allergic response when your mast cells are triggered. […] 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.

• #67 Mastocytosis: Types, Symptoms & Treatment

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

  Mastocytosis is an incurable disease. Different types of mastocytosis affect people in different ways. If youre living with mastocytosis, here are some general suggestions that may help: […] You’ll have regular checkups with your healthcare provider so they can monitor your symptoms and, if needed, adjust your treatment. Contact your provider any time you notice new symptoms or your symptoms are worse. […] Mastocytosis is a rare condition that happens when your body makes abnormal mast cells that multiply uncontrollably, affecting your skin and organs. Most people with mastocytosis manage its symptoms by knowing what triggers mastocytosis and by taking medication. People with this condition should also have self-injecting syringes of epinephrine at hand to treat severe allergic reactions. […] Cleveland Clinic can help you manage that with personalized treatment.

• #68 Systemic Mastocytosis: 6 Healthy Habits

  https://www.healthline.com/health/allergies/systemic-mastocytosis-healthy-habits

  Living with a chronic condition can be challenging. In addition to physical symptoms, mastocytosis is associated with psychological symptoms, including irritability, mood shifts, anxiety, and depression. […] Systemic mastocytosis is a lifelong condition that can present many challenges. In addition to following your treatment plan, following these six healthy habits can help you avoid potential triggers and better manage life with systemic mastocytosis.

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

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

  Systemic mastocytosis can be a complex disease. Understanding your condition is critical to ongoing care and prevention of complications. […] Follow your doctor’s recommended care and ongoing monitoring. […] Caring for a lifelong disorder such as systemic mastocytosis can be stressful and exhausting. Consider these strategies: […] You’ll need to make important decisions about care. Medical centers with specialty teams can offer you information about systemic mastocytosis, as well as advice and support, and can help you manage care. […] Ask for or accept help from family and friends when needed. Take time for your interests and activities. Counseling with a mental health professional may help with adjustment and coping.

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

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

  Systemic mastocytosis is a disease where too many abnormal mast cells (a type of white blood cell) build up in your skin and other organs. […] There’s no cure, but treatments can help you manage the condition. […] Consider reaching out to family and friends for support as you manage this disease. They can help you deal with the emotions and stress that come with the challenges of a long-term condition like 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. […] 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. […] If you find that the challenges of managing your disease are making you depressed or anxious, talk to your doctor. They can put you in touch with mental health professionals who are experts in treating these problems.

• #71 Advanced Systemic Mastocytosis: Symptoms, Causes, Diagnosis, and Treatment

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

  Finding a healthcare team that is experienced in treating advanced systemic mastocytosis is essential to managing it effectively, says Gotlib. You can ask your healthcare provider to refer you to a mastocytosis center of excellence, which can provide access to the latest treatments and possibly clinical trials. […] The most meaningful lifestyle change for advanced systemic mastocytosis is to avoid the triggers that can lead to inflammation and organ damage. […] Although current guidelines don’t suggest any special diet for people with advanced systemic mastocytosis, some experts recommended a low-histamine diet. […] The prognosis for individuals with advanced systemic mastocytosis depends on several factors, including the extent of mast cell accumulation, the organs involved, and the person’s overall health. […] Support is available. People can benefit from connecting with others who understand their experiences. Support groups, both in-person and online, provide a sense of community and can be a valuable resource for coping strategies, emotional support, and information about new treatments.

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

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

  Systemic mastocytosis can be a complex disease. Understanding your condition is critical to ongoing care and prevention of complications. […] Follow your doctor’s recommended care and ongoing monitoring. […] Caring for a lifelong disorder such as systemic mastocytosis can be stressful and exhausting. Consider these strategies: […] You’ll need to make important decisions about care. Medical centers with specialty teams can offer you information about systemic mastocytosis, as well as advice and support, and can help you manage care. […] Ask for or accept help from family and friends when needed. Take time for your interests and activities. Counseling with a mental health professional may help with adjustment and coping.

• #73 Systemic Mastocytosis, Version 2.2019, NCCN Clinical Practice Guidelines in Oncology in: Journal of the National Comprehensive Cancer Network Volume 16 Issue 12 (2018)

  https://jnccn.org/view/journals/jnccn/16/12/article-p1500.xml

  Enrollment in well-designed clinical trials investigating novel therapeutic strategies (eg, selective KIT D816 inhibitors) is encouraged to enable further advances. […] The use of bisphosphonates (with continued use of antihistamines) is recommended to resolve bone pain and improve vertebral bone mineral density (BMD). […] Denosumab, an anti-RANKL monoclonal antibody, has also been associated with significant increases in BMD at lumbar and femoral sites, decreases in bone turnover markers in serum. […] Allogeneic hematopoietic cell transplant (HCT) has been evaluated in patients with advanced SM, and the outcomes are significantly affected by the subtype of SM and the type of conditioning regimen used. […] The role of allogeneic HCT needs to be determined in a prospective trial. However, given the rarity of SM, no larger prospective trials of HCT have been initiated to confirm the role of allogeneic HCT.

• #74 Quest to find better treatments for rare blood cancer leads to new therapies | News Center

  https://med.stanford.edu/news/all-news/2022/03/systemic-mastocytosis-therapies.html

  „Overall, her quality of life was quite poor,” Gotlib said. […] Although a medication called imatinib, which is sold under the brand name Gleevec, has been approved for use in advanced SM, it is ineffective against the D816V mutation (although it may be useful in the rare SM patients who have normal KIT or other mutations in the gene). […] Gotlib suggested Worthen enroll in a phase-1 clinical trial he was leading of a drug called avapritinib, which has a mechanism of action similar to midostaurin’s but is less likely to cause severe side effects because it targets only the KIT protein. […] „Avapritinib is considerably more selective than midostaurin,” Gotlib said. „It was specifically designed to inhibit the KIT D816V mutation with very few other targets. Midostaurin was a big step forward in the treatment of advanced SM, but it hits several other targets, and avapritinib is more potent.”

• #75 Systemic Mastocytosis | Huntsman Cancer Institute | University of Utah Health

  https://healthcare.utah.edu/huntsmancancerinstitute/hematologic-cancers/systemic-mastocytosis

  Systemic mastocytosis is a rare disorder that happens when too many mast cells build up in parts of your body. […] Systemic mastocytosis originates in the bone marrow, and can happen in the skin, liver, spleen, stomach, small intestine, or colon. […] Your care team may include these specialists: Hematologists (Blood specialist doctors), pain and palliative care doctors, dermatologists, and doctors from other specialties, as needed. […] Our care teams may also include advanced care nurses, and physician assistants. Everyone on your care team talks with each other to plan and give treatment. They can talk to you about any questions or concerns you have about systemic mastocytosis.

• #76 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. […] The study highlighted the importance of a person-centred approach and the need to increase knowledge of the disease within primary care, to shorten this stressful and vulnerable time. […] The symptoms and signs can include, e.g., skin and gastrointestinal (GI) problems, cognitive disabilities, depression and life-threatening anaphylactic reactions. […] Since the disease can give rise to a variety of symptoms and signs and the time between symptom onset and diagnosis can be long, the person’s wellbeing can be severely affected.

• #77 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 time between symptom onset and diagnosis was perceived as difficult, with severe, complex and often unpredictable symptoms that the participants had to deal with on their own, often for many years. The healthcare deficiency in diagnosing and supporting these persons highlights the importance of a person-centred approach and the need to increase knowledge of SM within healthcare, in order to shorten this stressful and vulnerable time.

• #78 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 time between symptom onset and diagnosis was perceived as difficult, with severe, complex and often unpredictable symptoms that the participants had to deal with on their own, often for many years. The healthcare deficiency in diagnosing and supporting these persons highlights the importance of a person-centred approach and the need to increase knowledge of SM within healthcare, in order to shorten this stressful and vulnerable time.

• #79 Systemic mastocytosis – Overview of Information and Clinical Research

  https://clinicaltrials.eu/disease/systemic-mastocytosis/

  Treatment plans for systemic mastocytosis are highly personalized, taking into account the specific symptoms, affected organs, and the type of mastocytosis. This tailored approach ensures that patients receive the most effective care for their unique needs. […] Living with SM requires a comprehensive approach to managing symptoms and monitoring disease progression. Collaborating closely with healthcare providers, particularly those specializing in SM, is crucial for understanding one’s prognosis and effectively managing the disease. […] Personalized care plans are essential for effective management.

• #80 Duke Blood Cancer Center Named Mast Cell Diseases Center of Excellence | Duke Health

  https://www.dukehealth.org/blog/duke-blood-cancer-center-named-mast-cell-diseases-center-of-excellence

  The Duke Blood Cancer Center has been recognized as a Center of Excellence by the American Initiative in Mast Cell Diseases Network. This designation acknowledges the centers expertise in caring for people with systemic mastocytosis and related mast cell disorders. […] The Duke Blood Cancer Center is one of a handful of centers in the Southeast with the experience and capability to treat mast cell diseases. […] Having experts in multiple specialties — including hematology, allergy and immunology, dermatology, and oncology — is essential for helping people manage these symptoms, treating their disease, and improving their quality of life. […] In addition to patient care, Dr. Rein and her colleagues are actively involved in research on new treatments for systemic mastocytosis and other mast cell diseases. […] While there is currently no cure for systemic mastocytosis and related mast cell disorders, receiving comprehensive care at a Mast Cell Diseases Center of Excellence can help people with these conditions live a full and fulfilling life.

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