Clinical and epidemiological characteristics of the Philadelphia negative myeloproliferative neoplasms in Chile

被引:0
作者
Valladares, Ximena [1 ]
Benavente, Rafael [1 ]
Rojas, Christine [2 ]
Pena, Camila [1 ]
Valenzuela, Rodrigo [3 ]
Monardes, Virginia [3 ]
Lopez, Hernan [4 ]
Abarca, Marcelo [5 ]
Leon, Pilar [6 ]
Osorio, Rocio [7 ]
Perez, Erika [8 ]
Soto, Pablo [9 ]
La Rocca, Gabriel [10 ]
Cardemil, Daniela [11 ]
Torres, Vivianne [12 ]
Intriago, Marvila [13 ]
Elena Cabrera, Maria [1 ,14 ]
Soledad Undurraga, Maria [1 ]
机构
[1] Hosp Salvador, Secc Hematol, Santiago, Chile
[2] Hosp Gustavo Fricke, Secc Hematol, Vina Del Mar, Chile
[3] Hosp Salvador, Lab Biol Mol, Santiago, Chile
[4] Hosp Barros Luco, Secc Hematol, Santiago, Chile
[5] Hosp Sotero del Rio, Secc Hematol, Santiago, Chile
[6] Hosp Carlos Van Buren, Secc Hematol, Valparaiso, Chile
[7] Hosp Juan Noe, Secc Hematol, Arica, Chile
[8] Hosp Reg Vallenar Huasco, Secc Hematol, Vallenar, Chile
[9] Hosp Puerto Montt, Secc Hematol, Puerto Montt, Chile
[10] Hosp Reg Coyhaique, Sect Hematol, Coyhaique, Chile
[11] Hosp Clin Magallanes, Secc Hematol, Punta Arenas, Chile
[12] Hosp Reg Valdivia, Secc Hematol, Valdivia, Chile
[13] Hosp La Serena, Secc Hematol, La Serena, Chile
[14] Univ Chile, Dept Med Interna Oriente, Santiago, Chile
关键词
Janus Kinase 2; Polycythemia Vera; Primary Myelofibrosis; Thrombocythemia; Essential; WORLD-HEALTH-ORGANIZATION; ESSENTIAL THROMBOCYTHEMIA; POLYCYTHEMIA-VERA; MYELOID NEOPLASMS; CLASSIFICATION; TRANSFORMATION; REVISION; LEUKEMIA;
D O I
暂无
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Background: Philadelphia-negative myeloproliferative neoplasms (Ph-MPN) are chronic hematological disorders characterized by the overproduction of one or more mature myeloid blood cell lineages. Classical Ph-MPN are polycythemia vera (PV), essential thrombocytopenia (ET) and primary myelofibrosis (PMF). Aim: To assess the epidemiological, clinical and diagnostic characteristics of Ph-MPN in Chile. Material and Methods: Retrospective review of medical records of all patients referred as MPN from 2012 to 2017. Patients with (9;21) translocation were excluded. Results: Data of 462 cases with a median age of 69 years from 10 public hospitals was reviewed. ET was the most frequently Ph-MNP found. The incidence of Ph-MPN was 1.5 x 100.000 cases. The JAK2 V617F mutation study was performed in 96% of patients and only 30% had a bone marrow biopsy. Thrombotic events were observed in 29% of patients. Bleeding events were observed in 7%. Five-year overall survival was 87%. Conclusions: ET is the most frequent Ph-MPN. The mean incidence was lower than reported in the literature, in part because of a sub diagnosis.
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页码:1532 / 1538
页数:7
相关论文
共 18 条
[1]   Pathogenesis and Management of Thrombotic Disease in Myeloproliferative Neoplasms [J].
Arachchillage, Deepa R. J. ;
Laffan, Mike .
SEMINARS IN THROMBOSIS AND HEMOSTASIS, 2019, 45 (06) :604-611
[2]   The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia [J].
Arber, Daniel A. ;
Orazi, Attilio ;
Hasserjian, Robert ;
Thiele, Jurgen ;
Borowitz, Michael J. ;
Le Beau, Michelle M. ;
Bloomfield, Clara D. ;
Cazzola, Mario ;
Vardiman, James W. .
BLOOD, 2016, 127 (20) :2391-2405
[3]   Thrombosis in Philadelphia negative classical myeloproliferative neoplasms: a narrative review on epidemiology, risk assessment, and pathophysiologic mechanisms [J].
Ball, Somedeb ;
Thein, Kyaw Zin ;
Maiti, Abhishek ;
Nugent, Kenneth .
JOURNAL OF THROMBOSIS AND THROMBOLYSIS, 2018, 45 (04) :516-528
[4]   The 2016 WHO classification and diagnostic criteria for myeloproliferative neoplasms: document summary and in-depth discussion [J].
Barbui, Tiziano ;
Thiele, Jurgen ;
Gisslinger, Heinz ;
Kvasnicka, Hans Michael ;
Vannucchi, Alessandro M. ;
Guglielmelli, Paola ;
Orazi, Attilio ;
Tefferi, Ayalew .
BLOOD CANCER JOURNAL, 2018, 8 :15
[5]   Treatment-Related Risk Factors for Transformation to Acute Myeloid Leukemia and Myelodysplastic Syndromes in Myeloproliferative Neoplasms [J].
Bjorkholm, Magnus ;
Derolf, Asa R. ;
Hultcrantz, Malin ;
Kristinsson, Sigurdur Y. ;
Ekstrand, Charlotta ;
Goldin, Lynn R. ;
Andreasson, Bjorn ;
Birgegard, Gunnar ;
Linder, Olle ;
Malm, Claes ;
Markevarn, Berit ;
Nilsson, Lars ;
Samuelsson, Jan ;
Granath, Fredrik ;
Landgren, Ola .
JOURNAL OF CLINICAL ONCOLOGY, 2011, 29 (17) :2410-2415
[6]   Antithrombotic therapy for venous thromboembolism in myeloproliferative neoplasms [J].
De Stefano, Valerio ;
Finazzi, Guido ;
Barbui, Tiziano .
BLOOD CANCER JOURNAL, 2018, 8
[7]   Therapy for myeloproliferative neoplasms: when, which agent, and how? [J].
Geyer, Holly L. ;
Mesa, Ruben A. .
BLOOD, 2014, 124 (24) :3529-3537
[8]   Impact of the 2016 revised WHO criteria for myeloproliferative neoplasms, unclassifiable: Comparison with the 2008 version [J].
Iurlo, Alessandra ;
Gianelli, Umberto ;
Cattaneo, Daniele ;
Thiele, Juergen ;
Orazi, Attilio .
AMERICAN JOURNAL OF HEMATOLOGY, 2017, 92 (04) :E48-E51
[9]   Thrombotic and Bleeding Complications in Classical Myeloproliferative Neoplasms [J].
McMahon, Brandon ;
Stein, Brady L. .
SEMINARS IN THROMBOSIS AND HEMOSTASIS, 2013, 39 (01) :101-111
[10]   Epidemiology of myelofibrosis, essential thrombocythemia, and polycythemia vera in the European Union [J].
Moulard, Odile ;
Mehta, Jyotsna ;
Fryzek, Jon ;
Olivares, Robert ;
Iqbal, Usman ;
Mesa, Ruben A. .
EUROPEAN JOURNAL OF HAEMATOLOGY, 2014, 92 (04) :289-297