Universität zu Köln

Barbui, Tiziano, Thiele, Juergen, Ferrari, Alberto ORCID: 0000-0002-5939-8637, Vannucchi, Alessandro M. and Tefferi, Ayalew (2020). The new WHO classification for essential thrombocythemia calls for revision of available evidences. Blood Cancer J., 10 (2). LONDON: NATURE PUBLISHING GROUP. ISSN 2044-5385

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Abstract

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.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Barbui, Tiziano UNSPECIFIED UNSPECIFIED UNSPECIFIED Thiele, Juergen UNSPECIFIED UNSPECIFIED UNSPECIFIED Ferrari, Alberto UNSPECIFIED orcid.org/0000-0002-5939-8637 UNSPECIFIED Vannucchi, Alessandro M. UNSPECIFIED UNSPECIFIED UNSPECIFIED Tefferi, Ayalew UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-344051
DOI:	10.1038/s41408-020-0290-9
Journal or Publication Title:	Blood Cancer J.
Volume:	10
Number:	2
Date:	2020
Publisher:	NATURE PUBLISHING GROUP
Place of Publication:	LONDON
ISSN:	2044-5385
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language WORLD-HEALTH-ORGANIZATION; MYELOPROLIFERATIVE DISORDERS; RISK-FACTORS; POLYCYTHEMIA-VERA; MYELOID NEOPLASMS; MYELOFIBROSIS; THROMBOSIS; SURVIVAL; DIAGNOSIS; ANAGRELIDE Multiple languages Oncology; Hematology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/34405