Indolent systemic mastocytosisis a throw in of mastocytosis, a rare form of blood cancer.
Mastocytosis is linked with a mutation in the KIT D816V gene.
Indolent systemic mastocytosis is the most common and least severe subtype of systemic mastocytosis in adults.
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It can affect many organs, which may or may not include your skin.
Symptoms vary depending on where the extra mast cells develop.
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How Do Indolent Systemic Mastocytosis Symptoms Start?
When mast cells are activated, they release the compound histamine, which triggers an allergic response.
Normal mast cells reduce their activity when their work is done.
However, with systemicmastocytosis, abnormal mast cells operate in overdrive and exist in excess numbers.
Mediators like histamine cause inflammatory responses.
There are numerous potential triggers for mast cells to release mediators.
These triggers can include:
Indolent systemic mastocytosis symptoms start slowly.
Symptoms can be episodic and seemingly unexplained.
Since the disorder affects everyone differently, it can be hard to know which organs will be affected.
Many symptoms of ISM are often attributed to other causes before an accurate diagnosis is achieved.
In addition, factors such as disease rarity and provider unfamiliarity with ISM can contribute to a delayed diagnosis.
Symptoms of indolent systemic mastocytosis, therefore, move gradually.
ISM rarely worsens to a more advanced form.
It also is rare for symptoms of ISM rarely pose an immediate threat or interfere with organ function.
Indolent systemic mastocytosis treatment typically involves treating symptoms and reducing the risk of triggers.
Symptom Exacerbation/Onset During Flares/Attacks
Symptoms of indolent systemic mastocytosis can vary widely.
Summary
Indolent systemic mastocytosis is the most common subtype of systemic mastocytosis.
Normal mast cells release histamines and other mediators when triggered.
However, damaged mast cells build up in organ tissue and release too much histamine, causing symptoms.
Common triggers include insect stings, certain drugs, alcohol, and spicy foods.
While this form of the disease seldom becomes severe, symptoms can involve pain and discomfort.
Because this disease is rare and has symptoms that mimic other ailments, getting a diagnosis can be delayed.
Getting blood tests and biopsies and/or working with a specialist may help you get a faster diagnosis.
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