Literature DB >> 28280082

Bumpy road to the diagnosis of polycythaemia vera.

Rita de Sousa Gameiro1, Ana Rodrigues1, Fernando Martos Gonçalves1, José Pimenta da Graça1.   

Abstract

Polycythaemia vera (PV) is the most common myeloproliferative neoplasm, characterised by increased red cell mass that can present as an unspecified symptom or a thrombohaemorrhagic event. Its diagnosis is based on the presence of erythrocytosis, the identification of the Janus kinase 2 mutation and bone marrow aspirate or biopsy alterations. The challenge of this disease lies on the treatment approach. Its cornerstone is phlebotomy, but depending on the vascular risk, it can include cytoreductive agents, low-dose aspirin or even anticoagulation. We present the case of a 75-year-old woman, whose inaugural presentation of PV was an arterial peripheral occlusion followed by three recurrent events in the same arterial region and a pulmonary embolism. A phlebotomy was initially performed and, after the diagnosis was made, the patient was initiated on low-dose aspirin and anticoagulation with favourable outcome. 2017 BMJ Publishing Group Ltd.

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Year:  2017        PMID: 28280082      PMCID: PMC5353380          DOI: 10.1136/bcr-2016-218851

Source DB:  PubMed          Journal:  BMJ Case Rep        ISSN: 1757-790X


  17 in total

1.  Guidelines for the diagnosis, investigation and management of polycythaemia/erythrocytosis.

Authors:  Mary F McMullin; D Bareford; P Campbell; A R Green; Claire Harrison; Beverley Hunt; D Oscier; M I Polkey; J T Reilly; E Rosenthal; Kate Ryan; T C Pearson; Bridget Wilkins
Journal:  Br J Haematol       Date:  2005-07       Impact factor: 6.998

2.  Amendment to the guideline for diagnosis and investigation of polycythaemia/erythrocytosis.

Authors:  Mary F McMullin; John T Reilly; Peter Campbell; David Bareford; Anthony R Green; Claire N Harrison; Eibhlin Conneally; Kate Ryan
Journal:  Br J Haematol       Date:  2007-08-02       Impact factor: 6.998

3.  Vascular and neoplastic risk in a large cohort of patients with polycythemia vera.

Authors:  Roberto Marchioli; Guido Finazzi; Raffaele Landolfi; Jack Kutti; Heinz Gisslinger; Carlo Patrono; Raphael Marilus; Ana Villegas; Gianni Tognoni; Tiziano Barbui
Journal:  J Clin Oncol       Date:  2005-02-14       Impact factor: 44.544

4.  Elevated serum erythropoietin levels in patients with Budd-Chiari syndrome secondary to polycythemia vera: clinical implications for the role of JAK2 mutation analysis.

Authors:  Paul J Thurmes; David P Steensma
Journal:  Eur J Haematol       Date:  2006-07       Impact factor: 2.997

Review 5.  Thrombosis and haemorrhage in polycythaemia vera and essential thrombocythaemia.

Authors:  M A Elliott; A Tefferi
Journal:  Br J Haematol       Date:  2005-02       Impact factor: 6.998

6.  Leukocytosis as a major thrombotic risk factor in patients with polycythemia vera.

Authors:  Raffaele Landolfi; Leonardo Di Gennaro; Tiziano Barbui; Valerio De Stefano; Guido Finazzi; Rosamaria Marfisi; Gianni Tognoni; Roberto Marchioli
Journal:  Blood       Date:  2006-11-14       Impact factor: 22.113

7.  A prospective study of 338 patients with polycythemia vera: the impact of JAK2 (V617F) allele burden and leukocytosis on fibrotic or leukemic disease transformation and vascular complications.

Authors:  F Passamonti; E Rumi; D Pietra; C Elena; E Boveri; L Arcaini; E Roncoroni; C Astori; M Merli; S Boggi; C Pascutto; M Lazzarino; M Cazzola
Journal:  Leukemia       Date:  2010-07-15       Impact factor: 11.528

8.  Leukocytosis is a risk factor for recurrent arterial thrombosis in young patients with polycythemia vera and essential thrombocythemia.

Authors:  Valerio De Stefano; Tommaso Za; Elena Rossi; Alessandro M Vannucchi; Marco Ruggeri; Elena Elli; Caterina Micò; Alessia Tieghi; Rossella R Cacciola; Cristina Santoro; Giancarla Gerli; Paola Guglielmelli; Lisa Pieri; Francesca Scognamiglio; Francesco Rodeghiero; Enrico M Pogliani; Guido Finazzi; Luigi Gugliotta; Giuseppe Leone; Tiziano Barbui
Journal:  Am J Hematol       Date:  2010-02       Impact factor: 10.047

Review 9.  Thrombotic complications of myeloproliferative neoplasms: risk assessment and risk-guided management.

Authors:  A Casini; P Fontana; T P Lecompte
Journal:  J Thromb Haemost       Date:  2013-07       Impact factor: 5.824

10.  Recurrent thrombosis in patients with polycythemia vera and essential thrombocythemia: incidence, risk factors, and effect of treatments.

Authors:  Valerio De Stefano; Tommaso Za; Elena Rossi; Alessandro M Vannucchi; Marco Ruggeri; Elena Elli; Caterina Micò; Alessia Tieghi; Rossella R Cacciola; Cristina Santoro; Giancarla Gerli; Nicola Vianelli; Paola Guglielmelli; Lisa Pieri; Francesca Scognamiglio; Francesco Rodeghiero; Enrico M Pogliani; Guido Finazzi; Luigi Gugliotta; Roberto Marchioli; Giuseppe Leone; Tiziano Barbui
Journal:  Haematologica       Date:  2008-02-11       Impact factor: 9.941

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  1 in total

1.  A Case Report of Large Bilateral Pulmonary Embolism in a Patient With Janus Kinase 2 (JAK2) Positive Mutation.

Authors:  Zahid Khan; George Besis
Journal:  Cureus       Date:  2022-05-30
  1 in total

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