Indolent SM subtype associated with high disease burden

Though patients with ISM have a normal life expectancy, they need more health care visits and medications than the general population.

Patients with indolent systemic mastocytosis (SM) use more health care services and have more allergies than the general population, according to a recently published study in The Journal of Allergy and Clinical Immunology.

The authors aimed to assess the disease burden in patients with indolent SMIndolent SM A subtype of nonadvanced SM caused by the abnormal accumulation of mast cells in the bone marrow and other organs. Indolent SM accounts for around 90% of SM cases. diagnosed at the Mayo Clinic between 2005 and 2022. Researchers used natural language processing algorithms to compare their clinical data with those of healthy control individuals. 

Results showed that the median time between symptom onset and indolent SM diagnosis was approximately four years. Over 70% of patients with indolent SM experienced allergic episodes, and over 60% experienced diarrhea, difficulty breathing, fatigue and nausea. Around 24% of patients with SM had food allergies, compared to 7% of healthy controls. The incidence of osteoporosis was almost double in patients with indolent SM, affecting close to 70% of the population. 

Regarding healthcare system utilization, the authors observed that patients with indolent SM averaged approximately 7.5 outpatient visits per year compared to 2.5 visits among healthy control individuals. Furthermore, patients with indolent SM used between 14 and six medications regularly, while healthy controls used 0.5. 

“This study provides real-world insights quantifying ISM disease burden, demonstrating significant differences in healthcare utilization and allergy prevalence compared to matched controls,” the authors concluded.

Read more about SM signs and symptoms

About indolent SM clinical manifestations

The word “indolent” in indolent SM does not mean that patients will be symptom-free. In this context, indolent refers to the slow clinical course and low risk of progression associated with this SM subtype. Unlike advanced SMAdvanced SM In these subtypes of SM, mast cells begin to damage organs. Advanced SM includes the subtypes aggressive SM, mast cell leukemia and SM with an associated hematological neoplasm., patients with indolent SM have a normal life expectancy. However, symptoms associated with mast cell activationMast cell activation Describes when mast cells release histamine and other mediators into the blood stream in response to an allergen or other trigger. This leads to symptoms like fatigue, rash and, in severe cases, anaphylaxis. can significantly impact their quality of life.

Common symptoms associated with indolent SM include diarrhea, abdominal pain, cutaneous manifestations (flushing and itchiness), anaphylaxisAnaphylaxis A severe allergic reaction that can turn fatal without treatment. Patients with SM are at a higher risk of developing anaphylaxis. and neuropsychiatric symptoms. In contrast, patients with advanced SM experience symptoms associated with organ damage, including enlarged liver size (hepatosplenomegaly), anemia, liquid accumulation in the abdomen (ascites), enlarged lymph nodes, and weight loss.

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