Universität zu Köln

Barbui, T., Thiele, J., Vannucchi, A. M. and Tefferi, A. (2015). Rationale for revision and proposed changes of the WHO diagnostic criteria for polycythemia vera, essential thrombocythemia and primary myelofibrosis. Blood Cancer J., 5. LONDON: NATURE PUBLISHING GROUP. ISSN 2044-5385

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Abstract

The 2001/2008 World Health Organization (WHO)-based diagnostic criteria for polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF) were recently revised to accomodate new information on disease-specific mutations and underscore distinguishing morphologic features. In this context, it seems to be reasonable to compare first major diagnostic criteria of the former WHO classifications for myeloproliferative neoplasm (MPN) and then to focus on details that have been discussed and will be proposed for the upcoming revision of diagnostic guidelines. In PV, a characteristic bone marrow (BM) morphology was added as one of three major diagnostic criteria, which allowed lowering of the hemoglobin/hematocrit threshold for diagnosis, which is another major criterion, to 16.5 g/dl/49% in men and 16 g/dl/48% in women. The presence of a JAK2 mutation remains the third major diagnostic criterion in PV. Subnormal serum erythropoietin level is now the only minor criterion in PV and is used to capture JAK2-unmutated cases. In ET and PMF, mutations that are considered to confirm clonality and specific diagnosis now include CALR, in addition to JAK2 and MPL. Also in the 2015 discussed revision, overtly fibrotic PMF is clearly distinguished from early/prefibrotic PMF and each PMF variant now includes a separate list of diagnostic criteria. The main rationale for these changes was to enhance the distinction between so-called masked PV and JAK2-mutated ET and between ET and prefibrotic early PMF. The proposed changes also underscore the complementary role, as well as limitations of mutation analysis in morphologic diagnosis. On the other hand, discovery of new biological markers may probably be expected in the future to enhance discrimination of the different MPN subtypes in accordance with the histological BM patterns and corresponding clinical features.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Barbui, T. UNSPECIFIED UNSPECIFIED UNSPECIFIED Thiele, J. UNSPECIFIED UNSPECIFIED UNSPECIFIED Vannucchi, A. M. UNSPECIFIED UNSPECIFIED UNSPECIFIED Tefferi, A. UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-397989
DOI:	10.1038/bcj.2015.64
Journal or Publication Title:	Blood Cancer J.
Volume:	5
Date:	2015
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; CHRONIC IDIOPATHIC MYELOFIBROSIS; BONE-MARROW BIOPSY; JAK2 EXON-12 MUTATIONS; MYELOPROLIFERATIVE NEOPLASMS; CALRETICULIN MUTATIONS; HISTOLOGICAL CRITERIA; CLONAL HEMATOPOIESIS; JAK2(V617F) MUTATION; DISEASE PROGRESSION Multiple languages Oncology; Hematology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/39798