Universität zu Köln

Barbui, Tiziano, Thiele, Juergen, Gisslinger, Heinz, Finazzi, Guido, Carobbio, Alessandra ORCID: 0000-0001-9664-7084, Rumi, Elisa ORCID: 0000-0002-7572-9504, Randi, Maria Luigia, Betozzi, Irene, Vannucchi, Alessandro M., Pieri, Lisa ORCID: 0000-0003-1812-8072, Carrai, Valentina, Gisslinger, Bettina, Muellauer, Leonhard, Ruggeri, Marco, Rambaldi, Alessandro ORCID: 0000-0002-3739-7502 and Tefferi, Ayalew (2014). Masked polycythemia Vera (mPV): Results of an international study. Am. J. Hematol., 89 (1). S. 52 - 55. HOBOKEN: WILEY-BLACKWELL. ISSN 1096-8652

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Abstract

We examined the baseline features and clinical outcomes of 140 patients presenting with JAK2V617F positivity and a bone marrow morphology conforming with WHO criteria of polycythemia vera (PV), but a hemoglobin level of <18.5 g/dL in males (range 16.0-18.4) and <16.5 g/dL in females (range 15.0-16.4). This cohort operationally referred to as masked PV (mPV) was compared with 257 patients with overt PV and displayed male predominance, a more frequent history of arterial thrombosis and thrombocytosis. Incidence of thrombosis was similar between the two groups but mPV displayed significantly higher rates of progression to myelofibrosis and acute leukemia and inferior survival. In multivariable analysis mPV diagnosis was an independent predictor of poor survival along with age >65 years and leukocyte count >10 x 10(9)/L. Our data suggest that mPV is a heterogeneous myeloproliferative neoplasia and not necessarily an early/ pre-polycythemic form of classical PV that at onset in a small fraction of patients clinically may mimic essential thrombocythemia. On the other hand, the majority mPV may have a longer prodrome of undiagnosed PV or a disease biology akin to primary myelofibrosis-post PV myelofibrosis that could explain the worsening of outcome in comparison to overt/classical manifestations. Am. J. Hematol. 89:52-54, 2014. (c) 2013 Wiley Periodicals, Inc.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Barbui, Tiziano UNSPECIFIED UNSPECIFIED UNSPECIFIED Thiele, Juergen UNSPECIFIED UNSPECIFIED UNSPECIFIED Gisslinger, Heinz UNSPECIFIED UNSPECIFIED UNSPECIFIED Finazzi, Guido UNSPECIFIED UNSPECIFIED UNSPECIFIED Carobbio, Alessandra UNSPECIFIED orcid.org/0000-0001-9664-7084 UNSPECIFIED Rumi, Elisa UNSPECIFIED orcid.org/0000-0002-7572-9504 UNSPECIFIED Randi, Maria Luigia UNSPECIFIED UNSPECIFIED UNSPECIFIED Betozzi, Irene UNSPECIFIED UNSPECIFIED UNSPECIFIED Vannucchi, Alessandro M. UNSPECIFIED UNSPECIFIED UNSPECIFIED Pieri, Lisa UNSPECIFIED orcid.org/0000-0003-1812-8072 UNSPECIFIED Carrai, Valentina UNSPECIFIED UNSPECIFIED UNSPECIFIED Gisslinger, Bettina UNSPECIFIED UNSPECIFIED UNSPECIFIED Muellauer, Leonhard UNSPECIFIED UNSPECIFIED UNSPECIFIED Ruggeri, Marco UNSPECIFIED UNSPECIFIED UNSPECIFIED Rambaldi, Alessandro UNSPECIFIED orcid.org/0000-0002-3739-7502 UNSPECIFIED Tefferi, Ayalew UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-450986
DOI:	10.1002/ajh.23585
Journal or Publication Title:	Am. J. Hematol.
Volume:	89
Number:	1
Page Range:	S. 52 - 55
Date:	2014
Publisher:	WILEY-BLACKWELL
Place of Publication:	HOBOKEN
ISSN:	1096-8652
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language RED-CELL MASS; BONE-MARROW; ESSENTIAL THROMBOCYTHEMIA; ABSOLUTE ERYTHROCYTOSIS; DIAGNOSIS; CLASSIFICATION; MANAGEMENT; NEOPLASMS; IMPACT Multiple languages Hematology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/45098