icc_systemic_mastocytosis_criteria: ICC Diagnostic Criteria for Systemic Mastocytosis (2022)

In cliot: Clinical Indices and Outcomes Tools

ICC Diagnostic Criteria for Systemic Mastocytosis (2022)

Description

Evaluates the International Consensus Classification (ICC) diagnostic criteria for Systemic Mastocytosis (SM). Diagnosis requires either one Major criterion + at least one Minor criterion, OR at least three Minor criteria.

Usage

icc_systemic_mastocytosis_criteria(major_criterion_multifocal_infiltrates,
                                   minor_criterion_spindle_mast_cells,
                                   minor_criterion_kit_mutation,
                                   minor_criterion_cd25_expression,
                                   minor_criterion_tryptase_gt_20,
                                   minor_criterion_cd30_expression_bmm)

Arguments

major_criterion_multifocal_infiltrates	Numeric (0 or 1). Multifocal dense infiltrates of mast cells (>=15 mast cells in aggregates) detected in sections of bone marrow and/or other extracutaneous organ(s). (1 = Yes).
minor_criterion_spindle_mast_cells	Numeric (0 or 1). >25% of all mast cells are atypical cells (type I or type II) on bone marrow smears or are spindle-shaped in mast cell infiltrates detected on sections of bone marrow or other extracutaneous organ(s). (1 = Yes).
minor_criterion_kit_mutation	Numeric (0 or 1). KIT D816V mutation or other activating KIT mutation detected in bone marrow, blood, or other extracutaneous organ(s). (1 = Yes).
minor_criterion_cd25_expression	Numeric (0 or 1). Mast cells in bone marrow, blood, or other extracutaneous organ(s) express CD25 (with or without CD2). (1 = Yes).
minor_criterion_tryptase_gt_20	Numeric (0 or 1). Serum total tryptase persistently > 20 ng/mL (in absence of unrelated myeloid neoplasm). (1 = Yes).
minor_criterion_cd30_expression_bmm	Numeric (0 or 1). Mast cells in bone marrow display abnormal CD30 expression (Note: This is specific to Bone Marrow Mastocytosis/BMM context in some classifications, but included as a minor criterion in ICC updates for SM). (1 = Yes). *Note: MDCalc implementation often lists CD25/CD2/CD30 under the immunophenotype criterion. This function separates CD25 and CD30 if treated distinctly or groups them if standard.*

Value

A list containing:

Diagnosis	"Criteria Met" or "Criteria Not Met".
Major_Criteria_Met	Boolean indicating presence of the major criterion.
Minor_Criteria_Count	Count of minor criteria met.

References

Arber DA, Orazi A, Hasserjian RP, et al. International Consensus Classification of Myeloid Neoplasms and Acute Leukemias: integrating morphologic, clinical, and genomic data. Blood. 2022;140(11):1200-1228. doi:10.1182/blood.2022015850

Examples

# Example 1: Diagnosis Met (1 Major + 1 Minor)
# Major infiltrates (1), KIT mutation (1)
icc_systemic_mastocytosis_criteria(1, 0, 1, 0, 0, 0)

# Example 2: Diagnosis Met (3 Minor)
# No Major, Spindle cells (1), KIT mutation (1), CD25 (1)
icc_systemic_mastocytosis_criteria(0, 1, 1, 1, 0, 0)

cliot documentation built on Dec. 1, 2025, 1:06 a.m.