Survival, prognostic factors and rates of leukemic transformation in 381 untreated patients with MDS and del(5q): A multicenter study

被引：98

作者：

Germing, U. ^{[1
]}

Lauseker, M. ^{[2
]}

Hildebrandt, B. ^{[3
]}

Symeonidis, A. ^{[4
]}

Cermak, J. ^{[5
]}

Fenaux, P. ^{[6
]}

Kelaidi, C. ^{[6
]}

Pfeilstoecker, M. ^{[7
,8
]}

Noesslinger, T. ^{[7
,8
]}

Sekeres, M. ^{[9
]}

Maciejewski, J. ^{[9
]}

Haase, D. ^{[10
]}

Schanz, J. ^{[10
]}

Seymour, J. ^{[11
]}

Kenealy, M. ^{[11
]}

Weide, R. ^{[12
]}

Luebbert, M. ^{[13
]}

Platzbecker, U. ^{[14
]}

Valent, P. ^{[15
]}

Goetze, K. ^{[16
]}

Stauder, R. ^{[17
]}

Blum, S. ^{[18
]}

Kreuzer, K-A ^{[20
]}

Schlenk, R. ^{[19
]}

Ganser, A. ^{[21
]}

Hofmann, W-K ^{[22
]}

Aul, C. ^{[23
]}

Krieger, O. ^{[24
]}

Kuendgen, A. ^{[1
]}

Haas, R. ^{[1
]}

Hasford, J. ^{[2
]}

Giagounidis, A.

机构：

[1] Univ Dusseldorf, Dept Hematol Oncol & Clin Immunol, D-40225 Dusseldorf, Germany

[2] Univ Munich, Inst Med Informat Sci Biometry & Epidemiol, Munich, Germany

[3] Univ Dusseldorf, Inst Human Genet, D-40225 Dusseldorf, Germany

[4] Univ Hosp, Dept Internal Med, Div Hematol, Patras, Greece

[5] Inst Hematol & Blood Transfus, CR-12820 Prague, Czech Republic

[6] Hop Avicenne, Grp Francophone Myelodysplasies, F-93009 Bobigny, France

[7] Hanusch Hosp, Med Abt, Vienna, Austria

[8] Hanusch Hosp, L Boltzmann Inst Leukamieforsch & Hamatol, Vienna, Austria

[9] Cleveland Clin, Taussig Canc Inst, Dept Hematol Oncol & Blood Disorders, Cleveland, OH 44106 USA

[10] Univ Gottingen, Dept Hematol & Oncol, Gottingen, Germany

[11] Peter MacCallum Canc Inst, Dept Haematol, Melbourne, Vic 3000, Australia

[12] Praxisklin Hamatol & Onkol, Koblenz, Germany

[13] Univ Freiburg, Dept Hematol & Oncol, D-79106 Freiburg, Germany

[14] Univ Carl Gustav Carus, Dept Hematol & Oncol, Dresden, Germany

[15] Med Univ Vienna, Div Haematol & Hemostaseol, Dept Internal Med 1, Vienna, Austria

[16] Tech Univ Munich, Klinikum Rechts Isar, Div Hematol & Oncol, Dept Internal Med 3, D-8000 Munich, Germany

[17] Med Univ Innsbruck, Dept Internal Med Haematol & Oncol 5, Innsbruck, Austria

[18] CHU Vaudois, Serv Haematol, CH-1011 Lausanne, Switzerland

[19] Univ Ulm, Dept Internal Med 3, D-7900 Ulm, Germany

[20] Univ Cologne, Dept Internal Med 1, D-50931 Cologne, Germany

[21] Hannover Med Sch, Dept Hematol Hemostasis Oncol & Stem Cell Transpl, D-3000 Hannover, Germany

[22] Univ Mannheim, Dept Internal Med Haematol & Internal Oncol 3, Mannheim, Germany

[23] St Johannes Hosp Duisburg, Dept Hematol Oncol & Clin Immunol, Duisburg, Germany

[24] Hosp Elisabethinen, Dept Haematol Stem Cell Transplantat Hemostasel &, Linz, Austria

来源：

LEUKEMIA | 2012年 / 26卷 / 06期

关键词：

MDS and del(5q); prognosis; progression; transfusion need; MYELODYSPLASTIC-SYNDROME; SCORING SYSTEM; 5Q DELETION; LENALIDOMIDE; CLASSIFICATION; 5Q-SYNDROME; KARYOTYPE; EVOLUTION; FEATURES; IMPACT;

D O I：

10.1038/leu.2011.391

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

Myelodysplastic syndromes (MDS) with del(5q) are considered to have a benign course of the disease. In order to address the issue of the propensity of those patients to progress to acute myeloid leukemia (AML), data on 381 untreated patients with MDS and del(5q) characterized by low or intermediate I International Prognostic Scoring System (IPSS) risk score were collected from nine centers and registries. Median survival of the entire group was 74 months. Transfusion-dependent patients had a median survival of 44 months vs 97 months for transfusion-independent patients (P<0.0001). Transfusion need at diagnosis was the most important patient characteristic for survival. Of the 381 patients, 48 (12.6%) progressed to AML. The cumulative progression rate calculated using the Kaplan-Meier method was 4.9% at 2 years and 17.6% at 5 years. Factors associated with the risk of AML transformation were high-risk World Health Organization adapted Prognostic Scoring System (WPSS) score, marrow blast count >5% and red-cell transfusion dependency at diagnosis. In conclusion, patients with MDS and del(5q) are facing a considerable risk of AML transformation. More detailed cytogenetic and molecular studies may help to identify the patients at risk of progression.

引用

页码：1286 / 1292

页数：7

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