ICC_Primary_Myelofibrosis_Diagnostic_Criteria v.0.1.0

Bone marrow biopsy showing megakaryocytic proliferation and atypia, bone marrow fibrosis grade <2, increased age-adjusted BM cellularity, granulocytic proliferation, and decreased erythropoiesis

Bone marrow biopsy showing megakaryocytic proliferation and atypia, accompanied by reticulin and/or collagen fibrosis grades 2 or 3

JAK2, CALR, or MPL mutation or presence of another clonal marker

Absence of reactive bone marrow reticulin fibrosis

Absence of reactive myelofibrosis

Diagnostic criteria for ET, PV, BCR::ABL1-positive CML, myelodysplastic syndrome, or other myeloid neoplasms are not met

Anemia not attributed to a comorbid condition

Leukocytosis ≥11 × 109/L

Palpable splenomegaly

Lactate dehydrogenase level above the reference range

Leukoerythroblastosis

Evaluates recorded findings against the International Consensus Classification (ICC, 2022) diagnostic criteria to determine if the criteria for Primary Myelofibrosis (PMF) are met.

Kevin Arjona

kevin.arjona@cambio.se

To assess whether the combination of recorded major and minor criteria meets the threshold for diagnosing PMF according to the ICC guidelines, based on data recorded in the OBSERVATION.icc_pmf_criteria archetype.

The guideline checks if all 3 Major Criteria are met: Characteristic BM morphology Exclusion criteria met Required Mutation/Marker status present AND if at least 1 Minor Criterion is met: Anemia present Leukocytosis > 11 x 10⁹/L Palpable splenomegaly present LDH level above ULN Leukoerythroblastosis present The guideline outputs a determination of whether the overall ICC criteria for PMF diagnosis are met or not met.

Does not provide the final diagnosis, only determines if the criteria are formally met based on the inputs. Clinical correlation is still required.

Arber DA, Orazi A, Hasserjian RP, Borowitz MJ, Calvo KR, Kvasnicka HM, Wang SA, Bagg A, Barbui T, Branford S, Bueso-Ramos CE, Cortes JE, Dal Cin P, DiNardo CD, Dombret H, Duncavage EJ, Ebert BL, Estey EH, Facchetti F, Foucar K, Gangat N, Gianelli U, Godley LA, Gökbuget N, Gotlib J, Hellström-Lindberg E, Hobbs GS, Hoffman R, Jabbour EJ, Kiladjian JJ, Larson RA, Le Beau MM, Loh ML, Löwenberg B, Macintyre E, Malcovati L, Mullighan CG, Niemeyer C, Odenike OM, Ogawa S, Orfao A, Papaemmanuil E, Passamonti F, Porkka K, Pui CH, Radich JP, Reiter A, Rozman M, Rudelius M, Savona MR, Schiffer CA, Schmitt-Graeff A, Shimamura A, Sierra J, Stock WA, Stone RM, Tallman MS, Thiele J, Tien HF, Tzankov A, Vannucchi AM, Vyas P, Wei AH, Weinberg OK, Wierzbowska A, Cazzola M, Döhner H, Tefferi A. International Consensus Classification of Myeloid Neoplasms and Acute Leukemias: integrating morphologic, clinical, and genomic data. Blood. 2022 Sep 15;140(11):1200-1228. doi: 10.1182/blood.2022015850. PMID: 35767897; PMCID: PMC9479031.

openEHR-EHR-OBSERVATION.icc_pmf_criteria, openEHR-EHR-EVALUATION.icc_pmf_criteria_assessment

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