The new WHO classification for essential thrombocythemia calls for revision of available evidences
In: Blood Cancer Journal, Jg. 10 (2020), Heft 2, S. 1-6
Online
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Zugriff:
In the 2016 revised classification of myeloproliferative neoplasms pre-fibrotic primary myelofibrosis (pre-PMF) was recognized as a separate entity, distinct from essential thrombocythemia (ET). Owing that the majority of cases falling in the pre-PMF category were previously diagnosed as ET, one may question about the need to re-evaluate the results of epidemiologic, clinical, and molecular studies, and the results of clinical trials in the two entities. Based on a critical review of recently published studies, pre-PMF usually presents with a distinct clinical and hematological presentation and higher frequency of constitutional symptoms. JAK2V617F and CALR mutations in pre-PMF patients are superimposable to ET, whereas non-driver high-risk mutations are enriched in pre-PMF compared with ET. Thrombosis is not significantly different, whereas bleeding is more frequent in pre-PMF. Median survival is significantly shorter in pre-PMF and 10-year cumulative rates progression to overt myelofibrosis is 0–1% vs. 10–12%, and leukemic transformation is 1–2% vs. 2–6%, in ET and pre-fibrotic-PMF, respectively. Most patients fall in the lower prognostic IPSS group in which observation alone can be recommended. Patients at intermediate risk may require a symptom-driven treatment for anemia, splenomegaly or constitutional symptoms while cytoreductive drugs are indicated in the high-risk category.
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The new WHO classification for essential thrombocythemia calls for revision of available evidences
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Autor/in / Beteiligte Person: | Barbui, Tiziano ; Tefferi, Ayalew ; Thiele, Jürgen ; Ferrari, Alberto ; Vannucchi, Alessandro M. |
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Zeitschrift: | Blood Cancer Journal, Jg. 10 (2020), Heft 2, S. 1-6 |
Veröffentlichung: | Nature Publishing Group, 2020 |
Medientyp: | unknown |
ISSN: | 2044-5385 (print) |
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