One thousand patients with essential thrombocythemia: the Florence-CRIMM experience.
In: Blood Cancer Journal, Jg. 14 (2024-01-19), Heft 1, S. 1-12
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Zugriff:
We describe 1000 patients with essential thrombocythemia seen at the Center Research and Innovation of Myeloproliferative Neoplasms (CRIMM), Florence, Italy, between 1980 and 2023: median age 59 years (18–95), females 65%, JAK2/CALR/MPL-mutated 66%/19%/4%, triple-negative (TN) 11%. Extreme thrombocytosis (ExT, platelets ≥1000 × 10 9 /L) in 16%, leukocytosis (leukocytes >11 × 10 9 /L) in 16%, and at least one cardiovascular risk factor in 52% of cases. JAK2-mutated patients were older (median 62 years) and CALR-mutated and TN (53 years for both) younger (p < 0.001). Female gender clustered with TN (76%) and JAK2 (67%) vs CALR (46%) mutations (p < 0.001). ExT clustered with CALR (type-2 more than type-1), TN and MPL, and leukocytosis with JAK2 mutation (p < 0.001). In multivariable analysis, risk factors for arterial thrombosis-free survival were age ≥60 years (HR 2.0; p < 0.001) and JAK2 mutation (HR 1.3; p = 0.02) with borderline significance for male gender (p = 0.08) and cardiovascular risk factors (p = 0.08); for venous thrombosis-free survival, JAK2 mutation (HR 1.9; p = 0.03) with borderline significance for venous thrombosis history (p = 0.07); for overall survival, older age (p < 0.001), male gender (HR 1.9; p < 0.001), absolute neutrophil count (ANC) ≥ 8 × 10 9 /L (HR 1.8; p = 0.01), absolute lymphocyte count (ALC) < 1.7 × 10 9 /L (HR 1.2; p = 0.03); for myelofibrosis-free survival, CALR mutation (HR 2.7; p < 0.001, particularly for CALR type 1/1-like, HR 3.3) and MPL mutation (HR 3.9; p = 0.001); for leukemia-free survival, older age (p = 0.03). Cytoreductive therapy appeared to mitigate both venous (HR 0.3; p = 0.01) and arterial thrombosis (HR 4; p = 0.04); there was a trend for aspirin in preventing arterial thrombosis recurrence. The current study provides real-world observations in essential thrombocythemia, representing a valid source document for interpreting current literature and planning future studies. [ABSTRACT FROM AUTHOR]
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Titel: |
One thousand patients with essential thrombocythemia: the Florence-CRIMM experience.
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Autor/in / Beteiligte Person: | Loscocco, Giuseppe G. ; Gesullo, Francesca ; Capecchi, Giulio ; Atanasio, Alessandro ; Maccari, Chiara ; Mannelli, Francesco ; Vannucchi, Alessandro M. ; Guglielmelli, Paola |
Zeitschrift: | Blood Cancer Journal, Jg. 14 (2024-01-19), Heft 1, S. 1-12 |
Veröffentlichung: | 2024 |
Medientyp: | academicJournal |
ISSN: | 2044-5385 (print) |
DOI: | 10.1038/s41408-023-00968-7 |
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