A lower intensity of treatment may underlie the increased risk of thrombosis in young patients with masked polycythaemia vera

被引：47

作者：

Lussana, Federico ^{[1
]}

Carobbio, Alessandra ^{[2
]}

Randi, Maria L. ^{[3
]}

Elena, Chiara ^{[4
]}

Rumi, Elisa ^{[4
]}

Finazzi, Guido ^{[1
]}

Bertozzi, Irene ^{[3
]}

Pieri, Lisa ^{[5
]}

Ruggeri, Marco ^{[6
]}

Palandri, Francesca ^{[7
]}

Polverelli, Nicola ^{[7
]}

Elli, Elena ^{[8
]}

Tieghi, Alessia ^{[9
]}

Iurlo, Alessandra ^{[10
]}

Ruella, Marco ^{[11
]}

Cazzola, Mario ^{[4
]}

Rambaldi, Alessandro ^{[1
]}

Vannucchi, Alessandro M. ^{[5
]}

Barbui, Tiziano ^{[2
]}

机构：

[1] Azienda Osped Papa Giovanni XXIII, Haematol & Bone Marrow Transplant Unit, Bergamo, Italy

[2] Azienda Osped Papa Giovanni XXIII, Res Fdn, Bergamo, Italy

[3] Univ Padua, Dept Med DIMED, Padua, Italy

[4] Univ Pavia, Dept Haematol, IRCCS Policlin S Matteo, I-27100 Pavia, Italy

[5] Univ Florence, Azienda Osped Univ Careggi, Lab Congiunto MMPC, Dept Expt & Clin Med, Florence, Italy

[6] Osped Vicenza, Dept Haematol, Vicenza, Italy

[7] Inst Haematol Lorenzo & Ariosto Seragnoli, Dept Haematol & Oncol, Bologna, Italy

[8] San Gerardo Hosp, Div Haematol, Monza, Italy

[9] Azienda Osped Arcispedale Santa Maria Nuova IRCCS, Haematol Oncl Dept, Reggio Emilia, Italy

[10] Fdn IRCCS Ca Granda Osped Maggiore Policlin, Div Haematol, Milan, Italy

[11] Univ Turin, Grp Italiano Malattie EMatol Adulto GIMEMA, Haematol & Cell Therapy Dept, Turin, Italy

来源：

BRITISH JOURNAL OF HAEMATOLOGY | 2014年 / 167卷 / 04期

关键词：

polycythaemia vera; masked polycythaemia; essential thrombocythaemia; JAK2; mutation; thrombosis; ESSENTIAL THROMBOCYTHEMIA; MYELOPROLIFERATIVE NEOPLASMS; MUTATION STATUS; JAK2; CALR; CLASSIFICATION; REVISION; SUBTYPES;

D O I：

10.1111/bjh.13080

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

In patients who do not meet the World Health Organization (WHO) criteria for overt polycythaemia vera (PV), a diagnosis of masked PV (mPV) can be determined. A fraction of mPV patients may display thrombocytosis, thus mimicking essential thrombocythaemia (ET). No previous studies have examined clinical outcomes of mPV among young JAK2-mutated patients. We analysed a retrospective cohort of 538 JAK2-mutated patients younger than 40years, after a re-assessment of the diagnosis according to the haemoglobin threshold for mPV. In this cohort of patients, 97 (18%) met the WHO criteria for PV, 66 patients (12%) were classified as mPV and 375 (70%) as JAK2-mutated ET. Surprisingly, a significant difference in the incidence of thrombosis was found when comparing mPV versus overt PV patients (P=004). In multivariate analysis, the only factor accounting for the difference in the risk of thrombosis was the less frequent use of phlebotomies and cytoreduction in mPV patients compared to those with overt PV. Thus, we emphasize the need for the identification of mPV in young JAK2-mutated patients in order to optimize their treatments.

引用

页码：541 / 546

页数：6

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