_id

683a165cc782e11e38d1b38f

id

10556

title

International Consensus Classification (ICC) Diagnostic Criteria for Systemic Mastocytosis

full_title

International Consensus Classification (ICC) Diagnostic Criteria for Systemic Mastocytosis

short_title

ICC Diagnostic Criteria for Systemic Mastocytosis

med_description

Diagnoses systemic mastocytosis according to the International Consensus Classification (ICC).

short_description

Systemic mastocytosis diagnostic criteria.

slug

international-consensus-classification-icc-diagnostic-criteria-systemic-mastocytosis

description

The International Consensus Classification (ICC) Diagnostic Criteria for Systemic Mastocytosis diagnoses mast cell disorders.

keywords

International Consensus Classification (ICC) Diagnostic Criteria for Systemic Mastocytosis, ICC Diagnostic Criteria for Systemic Mastocytosis, Mast cell activation disorders, MCAD, bone marrow, mast cell, MC, mast cell activation, systemic mastocytosis, SM, MC clonality, serum baseline tryptase, sBt, indolent systemic mastocytosis, ISM, clonal MCAD, c-MCAD, nonclonal MCAD, nc-MCAD, clonal sMCAD, c-sMCAD, nonclonal sMCAD, nc-sMCAD, cutaneous mastocytosis, CM, MC sarcoma, MCS, smoldering SM, SSM, indolent SM, ISM, aggressive SM, ASM, SM-AHN, MC leukemia, MCL, MC activation syndrome, MCAS

complaint

[ "Abdominal Pain", "Diarrhea", "Dizziness", "Lymphadenopathy", "Myalgias", "Nausea", "Preventive Care/Screening", "Rash", "Shortness of Breath", "Syncope", "Vomiting", "Wheezing", "Bone Pain" ]

formula

The diagnosis of systemic mastocytosis (SM) is defined by the presence of the major criterion or, in its absence, by the presence of at least three minor criteria: In the absence of aKITmutation, particularly in cases with eosinophilia, the presence of tyrosine kinase gene fusions associated with myeloid/lymphoid neoplasm with eosinophilia and kinase gene fusion (M/LN-eo) must be excluded. Round-cell well-differentiated morphology can occur in a small subset of cases. In these cases, the mast cells are often negative for CD25 and CD2 but positive for CD30. To avoid “false negative” results, use of a high-sensitivity PCR assay for detection ofKITD816V mutation is recommended. If negative, exclusion ofKITmutation variants is strongly recommended in suspected SM.

evidence

measurements

[]

information

refrences

{ "Clinical Practice Guidelines": [], "Manufacturer Website": [], "Original/Primary Reference": [ { "href": "https://pubmed.ncbi.nlm.nih.gov/36214901/", "text": "Leguit RJ, Wang SA, George TI, Tzankov A, Orazi A. The international consensus classification of mastocytosis and related entities. Virchows Arch. 2023;482(1):99-112." } ], "Other References": [ { "href": "https://pubmed.ncbi.nlm.nih.gov/35767897/", "text": "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." } ], "Outcomes": [], "Validation": [], "Validations": [] }

pearls

usecase

Utilize these criteria when evaluating a patient for possible systemic mastocytosis.

reasons

Systemic mastocytosis is a heterogeneous disease with variable presentations. These criteria incorporate histopathologic and genetic information to guide diagnosis.

next_advice

Patients meeting the criteria for systemic mastocytosis should undergo further evaluation to determine the subtype of the disease, which is essential for guiding management decisions.

next_actions

next_management

diseases

[ "Asthma", "Autoimmune Disorders", "Osteoporosis", "Systemic Mastocytosis", "Anaphylaxis" ]

input_schema

[ { "inct": null, "option_fhir_rules": null, "subheading": "Major Criterion", "subheading_instructions": null, "type": "subheading" }, { "conditionality": null, "default": null, "label_en": "<p>Multifocal dense infiltrates of tryptase- and/or CD117-positive mast cells (&ge;15 mast cells in aggregates) detected in sections of bone marrow and/or other extracutaneous organ(s)</p>", "md_calc_info_concept": null, "name": "major", "option_fhir_rules": null, "optional": true, "options": [ { "label": "No", "value": 0 }, { "label": "Yes", "value": 1 } ], "show_points": false, "tips_en": "See <a href='#evidence'>Evidence</a> for more details.", "type": "toggle" }, { "inct": null, "option_fhir_rules": null, "subheading": "Minor Criteria", "subheading_instructions": null, "type": "subheading" }, { "conditionality": null, "default": null, "label_en": "<p>In bone marrow biopsy or in section of other extracutaneous organs, &gt;25% of mast cells are spindle-shaped or have an atypical immature morphology</p>", "md_calc_info_concept": null, "name": "minor1", "option_fhir_rules": null, "optional": true, "options": [ { "label": "No", "value": 0 }, { "label": "Yes", "value": 1 } ], "show_points": false, "tips_en": "See <a href='#evidence'>Evidence</a> for more details.", "type": "toggle" }, { "conditionality": null, "default": null, "label_en": "<p>Mast cells in bone marrow, peripheral blood, or other extracutaneous organs express CD25, CD2, and/or CD30, in addition to mast cell markers</p>", "md_calc_info_concept": null, "name": "minor2", "option_fhir_rules": null, "optional": true, "options": [ { "label": "No", "value": 0 }, { "label": "Yes", "value": 1 } ], "show_points": false, "tips_en": "", "type": "toggle" }, { "conditionality": null, "default": null, "label_en": "<p><em>KIT</em> D816V mutation or other activating <em>KIT</em> mutation detected in bone marrow, peripheral blood, or other extracutaneous organs</p>", "md_calc_info_concept": null, "name": "minor3", "option_fhir_rules": null, "optional": true, "options": [ { "label": "No", "value": 0 }, { "label": "Yes", "value": 1 } ], "show_points": false, "tips_en": "See <a href='#evidence'>Evidence</a> for more details.", "type": "toggle" }, { "conditionality": null, "default": null, "label_en": "<p>Elevated serum tryptase level, persistently &gt;20 ng/mL. In cases of <abbr title=\"SM with an associated myeloid neoplasm\">SM-AMN</abbr>, an elevated tryptase does not count as a SM minor criterion</p>", "md_calc_info_concept": null, "name": "minor4", "option_fhir_rules": null, "optional": true, "options": [ { "label": "No", "value": 0 }, { "label": "Yes", "value": 1 } ], "show_points": false, "tips_en": "", "type": "toggle" } ]

instructions

published

2024-08-08T14:29:58.276Z

purpose

[ "Diagnosis" ]

search_terms

[ "International Consensus Classification (ICC) Diagnostic Criteria for Systemic Mastocytosis", "ICC Diagnostic Criteria for Systemic Mastocytosis", "Mast cell activation disorders", "MCAD", "bone marrow", "mast cell", "MC", "mast cell activation", "systemic mastocytosis", "SM", "MC clonality", "serum baseline tryptase", "sBt", "indolent systemic mastocytosis", "ISM", "clonal MCAD", "c-MCAD", "nonclonal MCAD", "nc-MCAD", "clonal sMCAD", "c-sMCAD", "nonclonal sMCAD", "nc-sMCAD", "cutaneous mastocytosis", "CM", "MC sarcoma", "MCS", "smoldering SM", "SSM", "indolent SM", "ISM", "aggressive SM", "ASM", "SM-AHN", "MC leukemia", "MCL", "MC activation syndrome", "MCAS" ]

seo

{ "keywords_en": "International Consensus Classification (ICC) Diagnostic Criteria for Systemic Mastocytosis, ICC Diagnostic Criteria for Systemic Mastocytosis, Mast cell activation disorders, MCAD, bone marrow, mast cell, MC, mast cell activation, systemic mastocytosis, SM, MC clonality, serum baseline tryptase, sBt, indolent systemic mastocytosis, ISM, clonal MCAD, c-MCAD, nonclonal MCAD, nc-MCAD, clonal sMCAD, c-sMCAD, nonclonal sMCAD, nc-sMCAD, cutaneous mastocytosis, CM, MC sarcoma, MCS, smoldering SM, SSM, indolent SM, ISM, aggressive SM, ASM, SM-AHN, MC leukemia, MCL, MC activation syndrome, MCAS", "meta_description_en": "The International Consensus Classification (ICC) Diagnostic Criteria for Systemic Mastocytosis diagnoses mast cell disorders." }

specialty

[ "Allergy and Immunology", "Dermatology", "Family Practice", "Internal Medicine", "Primary Care" ]

departments

[ "Allergic", "Dermatologic", "Immunologic" ]

tags

[]

version_number

1

versions

[]

related

[ { "calcId": 10555, "short_title_en": "WHO Diagnostic Criteria for Systemic Mastocytosis", "slug": "world-health-organization-who-diagnostic-criteria-systemic-mastocytosis" }, { "calcId": 10557, "short_title_en": "MAPS Score", "slug": "mayo-alliance-prognostic-system-maps-score" }, { "calcId": 10554, "short_title_en": "REMA Score", "slug": "spanish-network-mastocytosis-rema-score" } ]

ismed

false

section

[ "whenToUseViewed", "whyUseViewed", "nextStepsViewed", "evidenceViewed" ]

cleaned_departments

[ "immunology", "dermatology", "immunology" ]

cleaned_use

[ "Utilize these criteria when evaluating a patient for possible systemic mastocytosis." ]

pub

false