Table I.
Diagnostic criteria in children with highly suspected systemic mastocytosis
| Patient number/sex | Major criterion of SM | Minor criteria of SM | Final diagnosis | ||||
|---|---|---|---|---|---|---|---|
| Tryptase (ng/ml) | KIT D816V mutation | Immunophenotype of MCs | Morphology of MCs | ||||
| PB | BM | ||||||
| 1/F | + | 30 | + | + | CD25+ | typical | ISM |
| 14/F | - | 29 | + | ND | CD25+ and CD2+ | atypical (>25%) | ISM |
| 15/F | + | 50 | + | + | CD25+ | typical | ISM |
| 17/F | - | 81 | + | ND | CD 25-, CD2- | typical | DCM |
| 21/F | - | 470 | + | + | CD25+ and CD2+ | atypical (7.8%) | SSM |
SM: systemic mastocytosis; PB: peripheral blood; BM: bone marrow; MCs: mast cells; ISM: indolent systemic mastocytosis; DCM: diffuse cutaneous mastocytosis; SSM: smoldering systemic mastocytosis.