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How to diagnose ISM

By Jen Wyatt Green

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May 27, 2026

Learning objective: After reading this article, learners will be able to identify current recommendations and best practices for the diagnosis of indolent systemic mastocytosis and implement them in clinical practice.


Do you know... Which of the following findings helps to distinguish ISM from more advanced subtypes of SM?

Systemic mastocytosis (SM) is a rare mast-cell neoplasm that can be difficult to recognize and is frequently underdiagnosed or misclassified in clinical practice.1 Advances in targeted therapies for SM have increased the importance of accurate diagnosis and disease classification for guiding clinical management.1 Indolent systemic mastocytosis (ISM) is the most common subtype of SM, accounting for ~70–80% of cases in adults.1 Here, we outline the latest diagnostic criteria for SM and highlight the diagnostic findings and key morphologic features that distinguish ISM from other subtypes. 

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This educational resource is independently supported by Blueprint Medicines. All content was developed by SES in collaboration with an expert steering committee. Funders were allowed no influence. 

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