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- Title
Clonal Megakaryocyte Dysplasia with Isolated Thrombocytosis Is a Distinct Myeloproliferative Neoplasm Phenotype.
- Authors
Barosi, Giovanni; Campanelli, Rita; Massa, Margherita; Catarsi, Paolo; Carolei, Adriana; Abbà, Carlotta; Villani, Laura; Magrini, Umberto; Gregato, Giuliana; Bertolini, Francesco; de Silvestri, Annalisa; Gale, Robert Peter; Rosti, Vittorio
- Abstract
Introduction: About 15% of people with a myeloproliferative neoplasm (MPN) are identified as MPN, unclassifiable using the 2016 WHO classification. Methods: We tested whether persons with platelet concentration ≥450 × 10E+9/L, bone marrow megakaryocyte morphology typical of prefibrotic/early myelofibrosis (pre-MF), and no minor criteria of pre-MF should be classified as a distinct MPN subtype, clonal megakaryocyte dysplasia with isolated thrombocytosis (CMD-IT). Results: 139 subjects meet these criteria who we compared with primary myelofibrosis (PMF) including 402 with pre-MF and 521 with overt myelofibrosis. CMD-IT subjects were more likely female and younger. They had lower frequencies of JAK2V617F compared with persons with PMF (55% vs. 70%; p < 0.001) and higher frequencies of CALR mutations (37% vs. 17%; p < 0.001). They also had lower frequency of variations associated with JAK2V617F susceptibility, JAK2 46/1 (35% vs. 47%; p = 0.021), and VEGFA rs3025039 (12% vs. 17%; p = 0.030). Subjects with CMD-IT had lower incidences of thrombotic events compared with those with pre-MF (9.7% vs. 26%; p < 0.001) and longer survival (median, not reached vs. 23 years; HR = 0.34 (0.10, 0.30); p < 0.001). Conclusion: Our data indicate CMD-IT is a distinct MPN subtype and should be included in the classification of myeloid neoplasms.
- Subjects
MYELOPROLIFERATIVE neoplasms; MYELOFIBROSIS; THROMBOCYTOSIS; DYSPLASIA; PHENOTYPES; BONE marrow
- Publication
Acta Haematologica, 2023, Vol 146, Issue 1, p14
- ISSN
0001-5792
- Publication type
Article
- DOI
10.1159/000527284