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Indolent Systemic Mastocytosis: Whats Different?
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.
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ISM is a unique subtype of systemicmastocytosis, distinguished by its slow-progressing (i.e., indolent) nature.
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.
Mast Cell Leukemia (MCL)
MCL is a rare and highly aggressive form of systemic mastocytosis.
Why Are Symptoms Indolent?
Consulting with specialists, such as allergists/immunologists, dermatologists, hematologists, and gastroenterologists, can help diagnose ISM.
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.
Prescription treatment is primarily aimed at controlling symptoms and preventing triggers that can lead to mast cell activation.
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).
They are often taken orally to manage gastrointestinal symptoms.
Corticosteroids
Topical steroids can reduce skin symptoms such as rashes and itching.
Summary
Indolent systemic mastocytosisis a subtype of systemic mastocytosis.
It is characterized by the slow accumulation of mast cells, primarily in the skin and digestive system.
Regular monitoring and lifestyle adjustments are crucial for patients with ISM to prevent complications and maintain well-being.
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Buonomo A, Nucera E, Criscuolo M.Treatment of indolent and advanced systemic mastocytosis.Mediterr J Hematol Infect Dis.
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