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    Leukemia research. 2018 Sep 6. pii: S0145-2126(18)30206-6. doi: 10.1016/j.leukres.2018.08.022
    Differing clinical features between Japanese and Caucasian patients with myelodysplastic syndromes: Analysis from the International Working Group for Prognosis of MDS.
    Miyazaki Y1,  Tuechler H2,  Sanz G3,  Schanz J4,  Garcia-Manero G5,  Solé F6,  Bennett JM7,  Bowen D8,  Fenaux P9,  Dreyfus F10,  Kantarjian H11,  Kuendgen A12,  Malcovati L13,  Cazzola M14,  Cermak J15,  Fonatsch C16,  Le Beau MM17,  Slovak ML18,  Santini V19,  Lübbert M20,  Maciejewski J21,  Machherndl-Spandl S22,  Magalhaes SMM23,  Pfeilstöcker M24,  Sekeres MA25,  Sperr WR26,  Stauder R27,  Tauro S28,  Valent P29,  Vallespi T30,  van de Loosdrecht AA31,  Germing U32,  Haase D33,  Greenberg PL34
    Author information
    1Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan. Electronic address: y-miyaza@nagasaki-u.ac.jp.
    2L. Boltzmann Institute for Leukemia Research, Vienna, Austria.
    3Hospital Universitario La Fe, Valencia, Spain.
    4University Medical Center, Clinics of Haematology and Medical Oncology, Göttingen, Germany.
    5The University of Texas MD Anderson Cancer Center, Houston, TX, United States.
    6Institut de Recerca contra la Leucèmia Josep Carreras, Barcelona, Spain.
    7James P. Wilmot Cancer Center, University of Rochester Medical Center, Rochester, NY, United States.
    8St James's University Hospital, Leeds, United Kingdom.
    9Hopital Avicenne, Assistance Publique-Hopitaux de Paris (AP-HP)/University of Paris XIII, Bobigny, France.
    10Hopital Cochin, AP-HP, University of Paris V, Paris, France.
    11The University of Texas MD Anderson Cancer Center, Houston, TX, United States.
    12Heinrich-Heine University Hospital, Dusseldorf, Germany.
    13Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Policlinico San Matteo and University of Pavia, Pavia, Italy.
    14Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Policlinico San Matteo and University of Pavia, Pavia, Italy.
    15Institute of Hematology and Blood Transfusion, Praha, Czech Republic.
    16Medical University of Vienna, Vienna, Austria.
    17University of Chicago Comprehensive Cancer Research Center, Chicago, IL, United States.
    18Department of Pathology, University of New Mexico, Albuquerque, NM, United States.
    19MDS Unit, Ematologia, AOU Careggi, Università degli Studi di Firenze, Firenze, Italy.
    20University of Freiburg Medical Center, Faculty of Medicine, Freiburg, Germany.
    21Cleveland Clinic, Cleveland, OH, United States.
    22Elisabethinen Hospital, Linz, Austria.
    23Federal University of Ceara, Fortaleza, Brazil.
    24Hanusch Hospital and Ludwig Boltzmann Cluster Oncology, Vienna, Austria.
    25Cleveland Clinic, Cleveland, OH, United States.
    26Department of Internal Medicine I, Division of Hematology & Hemostaseology and Ludwig Boltzmann Cluster Oncology, Medical University of Vienna, Austria.
    27University Hospital of Innsbruck, Innsbruck, Austria.
    28University of Dundee, Dundee, United Kingdom.
    29Department of Internal Medicine I, Division of Hematology & Hemostaseology and Ludwig Boltzmann Cluster Oncology, Medical University of Vienna, Austria.
    30Hospital Universitario Vall d'Hebron, Barcelona, Spain.
    31VU University Medical Center, Cancer Center Amsterdam, Amsterdam, The Netherlands.
    32Heinrich-Heine University Hospital, Dusseldorf, Germany.
    33University Medical Center, Clinics of Haematology and Medical Oncology, Göttingen, Germany.
    34Stanford Cancer Institute, Stanford, CA, United States.
    Abstract

    Clinical features of myelodysplastic syndromes (MDS) could be influenced by many factors, such as disease intrinsic factors (e.g., morphologic, cytogenetic, molecular), extrinsic factors (e.g, management, environment), and ethnicity. Several previous studies have suggested such differences between Asian and European/USA countries. In this study, to elucidate potential differences in primary untreated MDS between Japanese (JPN) and Caucasians (CAUC), we analyzed the data from a large international database collected by the International Working Group for Prognosis of MDS (300 and 5838 patients, respectively). JPN MDS were significantly younger with more severe cytopenias, and cytogenetic differences: less del(5q) and more +1/+1q, -1/del(1p), der(1;7), -9/del(9q), del(16q), and del(20q). Although differences in time to acute myeloid leukemia transformation did not occur, a significantly better survival in JPN was demonstrated, even after the adjustment for age and FAB subtypes, especially in lower, but not in higher prognostic risk categories. Certain clinical factors (cytopenias, blast percentage, cytogenetic risk) had different impact on survival and time to transformation to leukemia between the two groups. Although possible confounding events (e.g., environment, diet, and access to care) could not be excluded, our results indicated the existence of clinically relevant ethnic differences regarding survival in MDS between JPN and CAUC patients. The good performance of the IPSS-R in both CAUC and JP patients underlines that its common risk model is adequate for CAUC and JP.


    Copyright © 2018 The Authors. Published by Elsevier Ltd.. All rights reserved.

    KEYWORDS: Clinical features, Ethnicity, Karyotype, Myelodysplastic syndromes, Survival

    Publikations ID: 30219650
    Quelle: öffnen
     
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