* Advanced SM includes ASM, SM-AHNMD, and MCL.

+ indicates frequently seen; +/−, found in a subset of patients; −/+, rarely seen; −, not found.

† In most adult patients with ISM, UP-like skin lesions are detected. Those who have ISM but do not exhibit skin lesions are classified as (isolated) BM mastocytosis. In these cases, it is important to exclude advanced SM, a condition that is also presenting without cutaneous lesions in many cases.

‡ Circulating MCs are typically found in patients with classical MCL.