Criteria defining systemic mastocytosis (SM).
| * If at least 1 major and 1 one minor, or at least 3 minor criteria, are met, the diagnosis of SM can be established. | ||
| ** Activating mutations at codon 816; in most cases, c-kit D816V. | ||
| Abbreviations: MCs, mast cells; AHNMD, associated hematologic clonal non–mast cell lineage disease. | ||
| Major:* | Multifocal dense infiltrates of MCs in bone marrow or other extracutaneous organ(s) (>15 MCs in aggregate) | |
| Minor:* | a. | MCs in bone marrow or other extracutaneous organ(s) show an abnormal morphology (> 25%) |
| b. | c-kit mutation at codon 816** in extracutaneous organ(s) | |
| c. | MCs in bone marrow express CD2 and/or CD25 | |
| d. | Serum total tryptase > 20 ng/mL (does not count in patients who have AHNMD-type disease) | |
| * If at least 1 major and 1 one minor, or at least 3 minor criteria, are met, the diagnosis of SM can be established. | ||
| ** Activating mutations at codon 816; in most cases, c-kit D816V. | ||
| Abbreviations: MCs, mast cells; AHNMD, associated hematologic clonal non–mast cell lineage disease. | ||
| Major:* | Multifocal dense infiltrates of MCs in bone marrow or other extracutaneous organ(s) (>15 MCs in aggregate) | |
| Minor:* | a. | MCs in bone marrow or other extracutaneous organ(s) show an abnormal morphology (> 25%) |
| b. | c-kit mutation at codon 816** in extracutaneous organ(s) | |
| c. | MCs in bone marrow express CD2 and/or CD25 | |
| d. | Serum total tryptase > 20 ng/mL (does not count in patients who have AHNMD-type disease) | |