cliot (version 1.0.0)

icc_systemic_mastocytosis_criteria: 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)

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.

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.*

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)