How I treat polycythemia vera

被引:64
作者
Spivak, Jerry L. [1 ]
机构
[1] Johns Hopkins Univ, Sch Med, Dept Med, Div Hematol, Baltimore, MD 21205 USA
关键词
POST-ESSENTIAL THROMBOCYTHEMIA; V617F ALLELE BURDEN; TERM-FOLLOW-UP; MYELOPROLIFERATIVE NEOPLASMS; DRIVER-MUTATIONS; IRON-DEFICIENCY; BONE-MARROW; STEM-CELLS; LEUKEMIC TRANSFORMATION; PRIMARY MYELOFIBROSIS;
D O I
10.1182/blood.2018834044
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Since its discovery, polycythemia vera (PV) has challenged clinicians responsible for its diagnosis and management and scientists investigating its pathogenesis. As a clonal hematopoietic stem cell (HSC) disorder, PV is a neoplasm but its driver mutations result in overproduction of morphologically and functionally normal blood cells. PV arises in an HSC but it can present initially as isolated erythrocytosis, leukocytosis, thrombocytosis, or any combination of these together with splenomegaly or myelofibrosis, and it can take years for a true panmyelopathy to appear. PV shares the same JAK2 mutation as essential thrombocytosis and primary myelofibrosis, but erythrocytosis only occurs in PV. However, unlike secondary causes of erythrocytosis, in PV, the plasma volume is frequently expanded, masking the erythrocytosis and making diagnosis difficult if this essential fact is ignored. PV is not a monolithic disorder: female patients deregulate fewer genes and clinically behave differently than their male counterparts, while some PV patients are genetically predisposed to an aggressive clinical course. Nevertheless, based on what we have learned over the past century, most PV patients can lead long and productive lives. In this review, using clinical examples, I describe how I diagnose and manage PV in an evidence-based manner without relying on chemotherapy.
引用
收藏
页码:341 / 352
页数:12
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共 138 条
  • [1] Frequency and prognostic value of resistance/intolerance to hydroxycarbamide in 890 patients with polycythaemia vera
    Alvarez-Larran, Alberto
    Kerguelen, Ana
    Hernandez-Boluda, Juan C.
    Perez-Encinas, Manuel
    Ferrer-Marin, Francisca
    Barez, Abelardo
    Martinez-Lopez, Joaquin
    Cuevas, Beatriz
    Isabel Mata, M.
    Garcia-Gutierrez, Valentin
    Araguees, Pilar
    Montesdeoca, Sara
    Burgaleta, Carmen
    Caballero, Gonzalo
    Angel Hernandez-Rivas, J.
    Antonia Duran, M.
    Teresa Gomez-Casares, M.
    Besses, Carles
    [J]. BRITISH JOURNAL OF HAEMATOLOGY, 2016, 172 (05) : 786 - 793
  • [2] Assessment and prognostic value of the European LeukemiaNet criteria for clinicohematologic response, resistance, and intolerance to hydroxyurea in polycythemia vera
    Alvarez-Larran, Alberto
    Pereira, Arturo
    Cervantes, Francisco
    Arellano-Rodrigo, Eduardo
    Hernandez-Boluda, Juan-Carlos
    Ferrer-Marin, Francisca
    Angona, Anna
    Gomez, Montse
    Muina, Begona
    Guillen, Helga
    Teruel, Anabel
    Bellosillo, Beatriz
    Burgaleta, Carmen
    Vicente, Vicente
    Besses, Carles
    [J]. BLOOD, 2012, 119 (06) : 1363 - 1369
  • [3] Hydroxyurea does not appreciably reduce JAK2 V617F allele burden in patients with polycythemia vera or essential thrombocythemia
    Antonioli, Elisabetta
    Carobbio, Alessandra
    Pieri, Lisa
    Pancrazzi, Alessandro
    Guglielmelli, Paola
    Delaini, Federica
    Ponziani, Vanessa
    Bartalucci, Niccolo
    Tozzi, Lorenzo
    Bosi, Alberto
    Rambaldi, Alessandro
    Barbui, Tiziano
    Vannucchi, Alessandro M.
    [J]. HAEMATOLOGICA-THE HEMATOLOGY JOURNAL, 2010, 95 (08): : 1435 - 1438
  • [4] The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia
    Arber, Daniel A.
    Orazi, Attilio
    Hasserjian, Robert
    Thiele, Jurgen
    Borowitz, Michael J.
    Le Beau, Michelle M.
    Bloomfield, Clara D.
    Cazzola, Mario
    Vardiman, James W.
    [J]. BLOOD, 2016, 127 (20) : 2391 - 2405
  • [5] No correlation of intensity of phlebotomy regimen with risk of thrombosis in polycythemia vera: evidence from European Collaboration on Low-Dose Aspirin in Polycythemia Vera and Cytoreductive Therapy in Polycythemia Vera clinical trials
    Barbui, Tiziano
    Carobbio, Alessandra
    Ghirardi, Arianna
    Masciulli, Arianna
    Rambaldi, Alessandro
    Vannucchi, Alessandro M.
    [J]. HAEMATOLOGICA, 2017, 102 (06) : E219 - E221
  • [6] Revised response criteria for polycythemia vera and essential thrombocythemia: an ELN and IWG-MRT consensus project
    Barosi, Giovanni
    Mesa, Ruben
    Finazzi, Guido
    Harrison, Claire
    Kiladjian, Jean-Jacques
    Lengfelder, Eva
    McMullin, Mary F.
    Passamonti, Francesco
    Vannucchi, Alessandro M.
    Besses, Carlos
    Gisslinger, Heinz
    Samuelsson, Jan
    Verstovsek, Srdan
    Hoffman, Ronald
    Pardanani, Animesh
    Cervantes, Francisco
    Tefferi, Ayalew
    Barbui, Tiziano
    [J]. BLOOD, 2013, 121 (23) : 4778 - 4781
  • [7] Prognostic impact of bone marrow fibrosis in polycythemia vera: validation of the IWG-MRT study and additional observations
    Barraco, D.
    Cerquozzi, S.
    Hanson, C. A.
    Ketterling, R. P.
    Pardanani, A.
    Gangat, N.
    Tefferi, A.
    [J]. BLOOD CANCER JOURNAL, 2017, 7 : e538 - e538
  • [8] Fibrotic progression in Polycythemia vera is associated with early concomitant driver-mutations besides JAK2
    Bartels, S.
    Faisal, M.
    Buesche, G.
    Schlue, J.
    Kreipe, H.
    Lehmann, U.
    [J]. LEUKEMIA, 2018, 32 (02) : 556 - 558
  • [9] How I treat essential thrombocythemia
    Beer, Philip A.
    Erber, Wendy N.
    Campbell, Peter J.
    Green, Anthony R.
    [J]. BLOOD, 2011, 117 (05) : 1472 - 1482
  • [10] Two routes to leukemic transformation after a JAK2 mutation-positive myeloproliferative neoplasm
    Beer, Philip A.
    Delhommeau, Francois
    LeCouedic, Jean-Pierre
    Dawson, Mark A.
    Chen, Edwin
    Bareford, David
    Kusec, Rajko
    McMullin, Mary Frances
    Harrison, Claire N.
    Vannucchi, Alessandro M.
    Vainchenker, William
    Green, Anthony R.
    [J]. BLOOD, 2010, 115 (14) : 2891 - 2900