Universität zu Köln

Barbui, T., Thiele, J., Vannucchi, A. M. and Tefferi, A. (2014). Rethinking the diagnostic criteria of polycythemia vera. Leukemia, 28 (6). S. 1191 - 1196. LONDON: NATURE PUBLISHING GROUP. ISSN 1476-5551

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Abstract

The aim of this review is to critically address the validity and clinical applicability of three major diagnostic classification systems for polycythemia vera (PV), that is, those proposed by the Polycythemia Vera Study Group (PVSG), the British Committee for Standards in Haematology (BCSH) and the World Health Organization (WHO). Special focus is on which one of the three red cell parameters (hemoglobin-HB, hematocrit-HCT and red cell mass-RCM) should be used as the diagnostic hallmark of PV. The revised BCSH employed a persistently raised HCT level as the first diagnostic criterion in combination with the presence of a JAK2V617F mutation. On the other hand, the WHO classification used a raised HB value as a surrogate for increased RCM in association with molecular markers and for the first time, the bone marrow (BM) morphology was included as a minor criterion. Ongoing controversy and discussion regards the use of certain threshold values for HCT and HB as surrogates for RCM as well as the existence of prodromallatent disease, so-called masked PV (mPV). It has been shown that mPV can be recognized in patients not meeting the required HB or HCT threshold levels by both the WHO and BCSH criteria. These cases present with the same baseline clinical features as overt PV but present worsened survival. A critical reappraisal of the WHO criteria may suggest either to reduce the thresholds for HB or to consider HCT values as major diagnostic criterion, as in the BCSH, in association with JAK2V617F mutation. The clinical utility of using HCT as reference variable is supported also by results of clinical trials which explicitly recommend to use the HCT threshold for monitoring treatment. In questionable cases as in mPV, BM biopsy examinations should be mandated together with mutation analysis.

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:38-436844
DOI:	10.1038/leu.2013.380
Journal or Publication Title:	Leukemia
Volume:	28
Number:	6
Page Range:	S. 1191 - 1196
Date:	2014
Publisher:	NATURE PUBLISHING GROUP
Place of Publication:	LONDON
ISSN:	1476-5551
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language CHRONIC MYELOPROLIFERATIVE DISORDERS; JAK2 EXON-12 MUTATIONS; TYROSINE KINASE JAK2; BONE-MARROW BIOPSIES; RED-CELL MASS; ESSENTIAL THROMBOCYTHEMIA; PRIMARY MYELOFIBROSIS; CLASSIFICATION; FEATURES; RECOMMENDATIONS Multiple languages Oncology; Hematology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/43684