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. 2021 Jul 1;13(1):e2021046. doi: 10.4084/MJHID.2021.046

Figure 2. Flowchart for adult patients with suspected systemic mastocytosis (SM).

Figure 2

In adult patients with suspected SM without skin involvement, the basal serum tryptase level is an important initial screen parameter. In conditions of slightly increased tryptase level, the presence of the KIT D816V mutation may be investigated at first in peripheral blood (PB) provided that a highly sensitive test is warranted. If the D816V mutation is detected in PB cells, a BM examination is recommended. If the KIT D816V mutant is not detectable in the BM and symptoms are non-specific, the patient will be examined in the follow up. By contrast, if the D816V mutation is detected in the BM and SM criteria are fulfilled, the diagnosis of SM can be established. In adult patients with suspected mastocytosis and documented skin involvement, a BM investigation is required. Based on the presence or absence of criteria for SM, the final diagnosis will be SM or cutaneous mastocytosis (CM).