cliot (version 1.0.0)

who_sm_criteria: WHO Diagnostic Criteria for Systemic Mastocytosis (2016)

Description

Evaluates the 2016 World Health Organization (WHO) diagnostic criteria for Systemic Mastocytosis (SM). Diagnosis requires either one major criterion and at least one minor criterion, OR at least three minor criteria.

Usage

who_sm_criteria(bone_marrow_aggregates, aberrant_cd25_expression,
                kit_d816v_mutation, serum_tryptase_elevated,
                spindle_cells_abnormal)

Value

A list containing:

Diagnosis

"Criteria Met for Systemic Mastocytosis" or "Criteria Not Met".

Criteria_Status

A summary of the major criterion and the count of minor criteria met.

Arguments

bone_marrow_aggregates

Numeric (0 or 1). Major Criterion: Multifocal dense infiltrates of mast cells (>=15 mast cells in aggregates) detected in sections of bone marrow and/or other extracutaneous organs. (1 = Yes).

aberrant_cd25_expression

Numeric (0 or 1). Minor Criterion: Mast cells in bone marrow, blood, or other extracutaneous organs express CD25 (and/or CD2) in addition to normal mast cell markers. (1 = Yes).

kit_d816v_mutation

Numeric (0 or 1). Minor Criterion: Detection of KIT mutation at codon 816 in bone marrow, blood, or other extracutaneous organs. (1 = Yes).

serum_tryptase_elevated

Numeric (0 or 1). Minor Criterion: Serum total tryptase persistently exceeds 20 ng/mL (unless there is an associated clonal myeloid disorder). (1 = Yes).

spindle_cells_abnormal

Numeric (0 or 1). Minor Criterion: >25% of mast cells in bone marrow or other extracutaneous organ sections are spindle-shaped or have atypical morphology, or >25% of mast cells in bone marrow aspirate smears are immature or atypical. (1 = Yes).

References

Arber DA, Orazi A, Hasserjian R, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016;127(20):2391-2405. doi:10.1182/blood-2016-03-643544

Examples

# Example 1: Diagnosis Met (Major + 1 Minor)
# Aggregates present, KIT mutation present
who_sm_criteria(1, 0, 1, 0, 0)

# Example 2: Diagnosis Met (3 Minor)
# No aggregates, but CD25+, KIT+, Tryptase >20
who_sm_criteria(0, 1, 1, 1, 0)