Universität zu Köln

Barbui, Tiziano, Thiele, Juergen, Carobbio, Alessandra ORCID: 0000-0001-9664-7084, Gisslinger, Heinz, Finazzi, Guido, Rumi, Elisa ORCID: 0000-0002-7572-9504, Randi, Maria Luigia, Vannucchi, Alessandro M., Gisslinger, Bettina, Muellauer, Leonhard, Ruggeri, Marco, Rambaldi, Alessandro ORCID: 0000-0002-3739-7502 and Tefferi, Ayalew (2014). Masked polycythemia vera diagnosed according to WHO and BCSH classification. Am. J. Hematol., 89 (2). S. 199 - 203. HOBOKEN: WILEY-BLACKWELL. ISSN 1096-8652

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Abstract

Polycythemia vera (PV) is currently diagnosed by the World Health Organization (WHO) criteria regarding hemoglobin (HB) levels and JAK2V617F and related mutations or by the British Committee for Standards in Haematology (BCSH) guidelines predominantly based on hematocrit (HCT) values (>52% in men and >48% in women) in JAK2 mutated patients. We examined clinical features at diagnosis and outcome in 397 mutated PV patients showing a bone marrow (BM) morphology conforming with the WHO descriptions but including also cases with a HB level <18.5 g/dL in males (range 16.0-18.4) and <16.5 g/dL in females (range 15.0-16.4). These patients were regarded as masked PV (mPV) comprising 140 (35%) cases of our cohort. A comparison with the BCSH criteria based on HCT levels revealed a decrease of mPV patients to 59 (15%). In both classification systems, mPV patients were more males, presented more frequently with higher platelet counts, and increased BM reticulin fibrosis. A worsening of overall survival was documented in mPV patients in comparison with overt PV following the WHO (P=0.011) as well as the BCSH (P=0.0019) criteria. Risk factors for inferior survival in mPV were age >65 years and white blood cell count >15 x 10(9)/L. Without these risk factors mPV patients had the same survival as overt PV suggesting that a fraction of patients with HB lower than that required for WHO diagnosis should still be considered as overt PV. This study has established the existence of mPV by two different classification systems based on either HB or HCT threshold values. Am. J. Hematol. 89:199-202, 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 Carobbio, Alessandra UNSPECIFIED orcid.org/0000-0001-9664-7084 UNSPECIFIED Gisslinger, Heinz UNSPECIFIED UNSPECIFIED UNSPECIFIED Finazzi, Guido UNSPECIFIED UNSPECIFIED UNSPECIFIED Rumi, Elisa UNSPECIFIED orcid.org/0000-0002-7572-9504 UNSPECIFIED Randi, Maria Luigia UNSPECIFIED UNSPECIFIED UNSPECIFIED Vannucchi, Alessandro M. 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:38-446155
DOI:	10.1002/ajh.23617
Journal or Publication Title:	Am. J. Hematol.
Volume:	89
Number:	2
Page Range:	S. 199 - 203
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 ESSENTIAL THROMBOCYTHEMIA; BONE-MARROW; POLYCYTHAEMIA/ERYTHROCYTOSIS; HISTOPATHOLOGY; ERYTHROCYTOSIS; MYELOFIBROSIS; CRITERIA Multiple languages Hematology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/44615