Table of ContentsView AllTable of ContentsIndolent Systemic Mastocytosis: What’s Different?Why Are Symptoms Indolent?SymptomsTreatment GoalsAt-Home TreatmentPrescription TreatmentManagment
Table of ContentsView All
View All
Table of Contents
Indolent Systemic Mastocytosis: What’s Different?
Why Are Symptoms Indolent?
Symptoms
Treatment Goals
At-Home Treatment
Prescription Treatment
Managment
Unlike other types, ISM presents with a slower progression of symptoms, often leading to a delayed diagnosis.ISM also primarily affects the skin and digestive system, with a lesser impact on other organs.
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In systemic mastocytosis, too manymast cellsbuild up in certain tissues. These extra mast cells release more proteins, which causes a stronger immune response in those areas.
In ISM, mast cells accumulate in tissues but do not cause the severe damage seen in ASM.Patients with ISM typically experience skin lesions, such as urticaria pigmentosa, and gastrointestinal symptoms like nausea, diarrhea, and abdominal pain. These symptoms are less severe compared to ASM and can often be managed with appropriate treatment.
These variants differ in severity, progression, and impact on overall health. It is very important to get an accurate diagnosis and tailored treatment plans for each type:
Aggressive Systemic Mastocytosis (ASM)
ASM progresses quickly and involves the abnormal accumulation of mast cells in multiple organs, such as the liver, spleen, small intestines, and bones.Severe symptoms include organ dysfunction, severe bone pain, and bone fractures.ASM significantly impacts life expectancy and requires intensive treatment.
Smoldering Systemic Mastocytosis (SSM)
SSM is an intermediate form between ISM and ASM, with a moderate progression of symptoms. Symptoms are more severe than in ISM but less aggressive than in ASM, often involving bone marrow, an enlarged spleen, and an enlarged liver.
Mast Cell Leukemia (MCL)
MCL is a rare and highly aggressive form of systemic mastocytosis. Rapidly progressing symptoms include high levels of mast cells in the blood, severe organ dysfunction, and bone marrow failure.MCL has a poorer prognosis and requires aggressive treatment.
Systemic Mastocytosis With an Associated Hematologic Neoplasm (SM-AHN)
SM-AHN is a rare condition in which abnormal mast cells accumulate in various tissues alongside other blood-related disorders, such as acute myeloid leukemia (AML).
The indolent nature of ISM symptoms can be attributed to the slower accumulation and activation of mast cells in tissues. ISM has a lower effect on mast cells, and life expectancy is similar to that of the general population. With ISM, there is also about a 3% to 5% risk of progression to a more advanced type of mastocytosis.
Systemic Mastocytosis: Chronic Characteristics and Symptom Variability
How to Tell if Symptoms Could Be Indolent Systemic Mastocytosis
Identifying ISM can be challenging due to its slow symptom progression.
Common symptoms include:
Keeping a detailed diary of symptoms, including their frequency, duration, and triggers, can provide valuable information for healthcare providers.
Consulting with specialists, such as allergists/immunologists, dermatologists, hematologists, and gastroenterologists, can help diagnose ISM. These specialists can perform tests to identify abnormal mast cells, including:
Goal of Indolent Systemic Mastocytosis Treatment
Symptom Soothing at Home
Managing ISM symptoms at home involves various strategies to reduce triggers and alleviate discomfort. Using gentle skin care products and avoiding harsh chemicals can help prevent skin irritation or urticaria pigmentosa.
Various triggers that prompt mast cells to release histamine and other chemicals can exacerbate mastocytosis. Identifying and avoiding allergens, stress, and other triggers that can exacerbate symptoms is important. These triggers can vary among individuals, but common ones include:
Avoiding these triggers and managing exposure can help individuals with mastocytosis maintain better control over their symptoms and improve their quality of life.
Prescribed Indolent Systemic Mastocytosis Treatment
Though ISM generally has a better prognosis compared to other forms of systemic mastocytosis, managing its symptoms is crucial for improving patients' quality of life. Prescription treatment is primarily aimed at controlling symptoms and preventing triggers that can lead to mast cell activation. Medical treatment for ISM may include various prescribed medications, some of which include:
H1 Antihistamines
These are commonly prescribed to alleviate symptoms such as itching, flushing, and hives. Examples include cetirizine (Zyrtec) and loratadine (Claritin). These medications are typically taken at bedtime, especially when symptoms interfere with sleeping quality.
H2 Antihistamines
These help reduce gastrointestinal symptoms like stomach pain, nausea, and diarrhea. Examples include ranitidine (Zantac) and famotidine (Pepcid).
Proton Pump Inhibitors (PPIs)
These medications, such as omeprazole (Prilosec OTC) and lansoprazole (Prevacid), reduce stomach acid production and can help alleviate gastrointestinal symptoms, particularly in patients with acid-related issues.
Mast Cell Stabilizers
These medications help stabilize mast cells and prevent the release of histamine and other inflammatory mediators. They are often taken orally to manage gastrointestinal symptoms.
Corticosteroids
Topical steroids can reduce skin symptoms such as rashes and itching. Oral or injected corticosteroids may be prescribed for severe symptoms, but long-term use is generally avoided due to potential side effects.
Leukotriene Inhibitors
Epinephrine
Patients at risk of severe allergic reactions or anaphylaxis are often prescribed an epinephrine auto-injector (EpiPen) for emergency use.
How to Manage Indolent Systemic Mastocytosis
Managing ISM involves ongoing measures and coping strategies such as:
It is also a good idea to keep emergency medications, such as epinephrine auto-injectors, with you in an emergency.
Summary
Treatment focuses on managing symptoms and improving quality of life through medications that stabilize mast cells and alleviate associated symptoms. Regular monitoring and lifestyle adjustments are crucial for patients with ISM to prevent complications and maintain well-being.
12 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Genetic and Rare Diseases Information Center.Mastocytosis.American Academy of Allergy, Asthma & Immunology.Systemic mastocytosis.MedlinePlus.Systemic mastocytosis.Leukemia & Lymphoma Society.Mastocytosis.Orphanet.Indolent systemic mastocytosis.National Cancer Institute.Aggressive systemic mastocytosis.National Organization for Rare Disorders.Mastocytosis - video.American Society of Hematology.Mast cell leukemia.National Cancer Institute.Systemic mastocytosis with associated hematologic neoplasm.National Organization for Rare Disorders.Mastocytosis.Buonomo A, Nucera E, Criscuolo M.Treatment of indolent and advanced systemic mastocytosis.Mediterr J Hematol Infect Dis. 2022;14(1):e2022040. doi:10.4084/MJHID.2022.040Buonomo A, Nucera E, Criscuolo M. (2022)Treatment of indolent and advances systemic mastocytosis: systemic mastocytosis treatment.Mediterranean Journal of Hematology and Infectious Diseases. 14(1), p. e2022040. doi: 10.4084/MJHID.2022.040.
12 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Genetic and Rare Diseases Information Center.Mastocytosis.American Academy of Allergy, Asthma & Immunology.Systemic mastocytosis.MedlinePlus.Systemic mastocytosis.Leukemia & Lymphoma Society.Mastocytosis.Orphanet.Indolent systemic mastocytosis.National Cancer Institute.Aggressive systemic mastocytosis.National Organization for Rare Disorders.Mastocytosis - video.American Society of Hematology.Mast cell leukemia.National Cancer Institute.Systemic mastocytosis with associated hematologic neoplasm.National Organization for Rare Disorders.Mastocytosis.Buonomo A, Nucera E, Criscuolo M.Treatment of indolent and advanced systemic mastocytosis.Mediterr J Hematol Infect Dis. 2022;14(1):e2022040. doi:10.4084/MJHID.2022.040Buonomo A, Nucera E, Criscuolo M. (2022)Treatment of indolent and advances systemic mastocytosis: systemic mastocytosis treatment.Mediterranean Journal of Hematology and Infectious Diseases. 14(1), p. e2022040. doi: 10.4084/MJHID.2022.040.
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Genetic and Rare Diseases Information Center.Mastocytosis.American Academy of Allergy, Asthma & Immunology.Systemic mastocytosis.MedlinePlus.Systemic mastocytosis.Leukemia & Lymphoma Society.Mastocytosis.Orphanet.Indolent systemic mastocytosis.National Cancer Institute.Aggressive systemic mastocytosis.National Organization for Rare Disorders.Mastocytosis - video.American Society of Hematology.Mast cell leukemia.National Cancer Institute.Systemic mastocytosis with associated hematologic neoplasm.National Organization for Rare Disorders.Mastocytosis.Buonomo A, Nucera E, Criscuolo M.Treatment of indolent and advanced systemic mastocytosis.Mediterr J Hematol Infect Dis. 2022;14(1):e2022040. doi:10.4084/MJHID.2022.040Buonomo A, Nucera E, Criscuolo M. (2022)Treatment of indolent and advances systemic mastocytosis: systemic mastocytosis treatment.Mediterranean Journal of Hematology and Infectious Diseases. 14(1), p. e2022040. doi: 10.4084/MJHID.2022.040.
Genetic and Rare Diseases Information Center.Mastocytosis.
American Academy of Allergy, Asthma & Immunology.Systemic mastocytosis.
MedlinePlus.Systemic mastocytosis.
Leukemia & Lymphoma Society.Mastocytosis.
Orphanet.Indolent systemic mastocytosis.
National Cancer Institute.Aggressive systemic mastocytosis.
National Organization for Rare Disorders.Mastocytosis - video.
American Society of Hematology.Mast cell leukemia.
National Cancer Institute.Systemic mastocytosis with associated hematologic neoplasm.
National Organization for Rare Disorders.Mastocytosis.
Buonomo A, Nucera E, Criscuolo M.Treatment of indolent and advanced systemic mastocytosis.Mediterr J Hematol Infect Dis. 2022;14(1):e2022040. doi:10.4084/MJHID.2022.040
Buonomo A, Nucera E, Criscuolo M. (2022)Treatment of indolent and advances systemic mastocytosis: systemic mastocytosis treatment.Mediterranean Journal of Hematology and Infectious Diseases. 14(1), p. e2022040. doi: 10.4084/MJHID.2022.040.
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