t(6;9)(p22; q34)/DEK-NUP214-rearranged pediatric myeloid leukemia: an international study of 62 patients

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t(6;9)(p22; q34)/DEK-NUP214-rearranged pediatric myeloid leukemia: an international study of 62 patients. / Sandahl, Julie Damgaard; Coenen, Eva A.; Forestier, Erik; Harbott, Jochen; Johansson, Bertil; Kerndrup, Gitte; Adachi, Souichi; Auvrignon, Anne; Beverloo, H. Berna; Cayuela, Jean-Michel; Chilton, Lucy; Fornerod, Maarten; de Haas, Valerie; Harrison, Christine J.; Inaba, Hiroto; Kaspers, Gertjan J. L.; Liang, Der-Cherng; Locatelli, Franco; Masetti, Riccardo; Perot, Christine; Raimondi, Susana C.; Reinhardt, Katarina; Tomizawa, Daisuke; von Neuhoff, Nils; Zecca, Marco; Zwaan, C. Michel; van den Heuvel-Eibrink, Marry M.; Hasle, Henrik.

In: Haematologica, Vol. 99, No. 5, 2014, p. 865-872.

Research output: Contribution to journalArticle

Harvard

Sandahl, JD, Coenen, EA, Forestier, E, Harbott, J, Johansson, B, Kerndrup, G, Adachi, S, Auvrignon, A, Beverloo, HB, Cayuela, J-M, Chilton, L, Fornerod, M, de Haas, V, Harrison, CJ, Inaba, H, Kaspers, GJL, Liang, D-C, Locatelli, F, Masetti, R, Perot, C, Raimondi, SC, Reinhardt, K, Tomizawa, D, von Neuhoff, N, Zecca, M, Zwaan, CM, van den Heuvel-Eibrink, MM & Hasle, H 2014, 't(6;9)(p22; q34)/DEK-NUP214-rearranged pediatric myeloid leukemia: an international study of 62 patients', Haematologica, vol. 99, no. 5, pp. 865-872. https://doi.org/10.3324/haematol.2013.098517

APA

CBE

Sandahl JD, Coenen EA, Forestier E, Harbott J, Johansson B, Kerndrup G, Adachi S, Auvrignon A, Beverloo HB, Cayuela J-M, Chilton L, Fornerod M, de Haas V, Harrison CJ, Inaba H, Kaspers GJL, Liang D-C, Locatelli F, Masetti R, Perot C, Raimondi SC, Reinhardt K, Tomizawa D, von Neuhoff N, Zecca M, Zwaan CM, van den Heuvel-Eibrink MM, Hasle H. 2014. t(6;9)(p22; q34)/DEK-NUP214-rearranged pediatric myeloid leukemia: an international study of 62 patients. Haematologica. 99(5):865-872. https://doi.org/10.3324/haematol.2013.098517

MLA

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Author

Sandahl, Julie Damgaard ; Coenen, Eva A. ; Forestier, Erik ; Harbott, Jochen ; Johansson, Bertil ; Kerndrup, Gitte ; Adachi, Souichi ; Auvrignon, Anne ; Beverloo, H. Berna ; Cayuela, Jean-Michel ; Chilton, Lucy ; Fornerod, Maarten ; de Haas, Valerie ; Harrison, Christine J. ; Inaba, Hiroto ; Kaspers, Gertjan J. L. ; Liang, Der-Cherng ; Locatelli, Franco ; Masetti, Riccardo ; Perot, Christine ; Raimondi, Susana C. ; Reinhardt, Katarina ; Tomizawa, Daisuke ; von Neuhoff, Nils ; Zecca, Marco ; Zwaan, C. Michel ; van den Heuvel-Eibrink, Marry M. ; Hasle, Henrik. / t(6;9)(p22; q34)/DEK-NUP214-rearranged pediatric myeloid leukemia: an international study of 62 patients. In: Haematologica. 2014 ; Vol. 99, No. 5. pp. 865-872.

RIS

TY - JOUR

T1 - t(6;9)(p22; q34)/DEK-NUP214-rearranged pediatric myeloid leukemia: an international study of 62 patients

AU - Sandahl, Julie Damgaard

AU - Coenen, Eva A.

AU - Forestier, Erik

AU - Harbott, Jochen

AU - Johansson, Bertil

AU - Kerndrup, Gitte

AU - Adachi, Souichi

AU - Auvrignon, Anne

AU - Beverloo, H. Berna

AU - Cayuela, Jean-Michel

AU - Chilton, Lucy

AU - Fornerod, Maarten

AU - de Haas, Valerie

AU - Harrison, Christine J.

AU - Inaba, Hiroto

AU - Kaspers, Gertjan J. L.

AU - Liang, Der-Cherng

AU - Locatelli, Franco

AU - Masetti, Riccardo

AU - Perot, Christine

AU - Raimondi, Susana C.

AU - Reinhardt, Katarina

AU - Tomizawa, Daisuke

AU - von Neuhoff, Nils

AU - Zecca, Marco

AU - Zwaan, C. Michel

AU - van den Heuvel-Eibrink, Marry M.

AU - Hasle, Henrik

PY - 2014

Y1 - 2014

N2 - Acute myeloid leukemia with t(6; 9)(p22; q34) is listed as a distinct entity in the 2008 World Health Organization classification, but little is known about the clinical implications of t(6; 9)-positive myeloid leukemia in children. This international multicenter study presents the clinical and genetic characteristics of 62 pediatric patients with t(6; 9)/DEKNUP214-rearranged myeloid leukemia; 54 diagnosed as having acute myeloid leukemia, representing <1% of all childhood acute myeloid leukemia, and eight as having myelodysplastic syndrome. The t(6; 9)/DEK-NUP214 was associated with relatively late onset (median age 10.4 years), male predominance (sex ratio 1.7), French-American-British M2 classification (54%), myelodysplasia (100%), and FLT3-ITD (42%). Outcome was substantially better than previously reported with a 5-year event-free survival of 32%, 5-year overall survival of 53%, and a 5-year cumulative incidence of relapse of 57%. Hematopoietic stem cell transplantation in first complete remission improved the 5-year event-free survival compared with chemotherapy alone (68% versus 18%; P<0.01) but not the overall survival (68% versus 54%; P=0.48). The presence of FLT3-ITD had a non-significant negative effect on 5-year overall survival compared with non-mutated cases (22% versus 62%; P=0.13). Gene expression profiling showed a unique signature characterized by significantly higher expression of EYA3, SESN1, PRDM2/RIZ, and HIST2H4 genes. In conclusion, t(6; 9)/DEK-NUP214 represents a unique subtype of acute myeloid leukemia with a high risk of relapse, high frequency of FLT3-ITD, and a specific gene expression signature.

AB - Acute myeloid leukemia with t(6; 9)(p22; q34) is listed as a distinct entity in the 2008 World Health Organization classification, but little is known about the clinical implications of t(6; 9)-positive myeloid leukemia in children. This international multicenter study presents the clinical and genetic characteristics of 62 pediatric patients with t(6; 9)/DEKNUP214-rearranged myeloid leukemia; 54 diagnosed as having acute myeloid leukemia, representing <1% of all childhood acute myeloid leukemia, and eight as having myelodysplastic syndrome. The t(6; 9)/DEK-NUP214 was associated with relatively late onset (median age 10.4 years), male predominance (sex ratio 1.7), French-American-British M2 classification (54%), myelodysplasia (100%), and FLT3-ITD (42%). Outcome was substantially better than previously reported with a 5-year event-free survival of 32%, 5-year overall survival of 53%, and a 5-year cumulative incidence of relapse of 57%. Hematopoietic stem cell transplantation in first complete remission improved the 5-year event-free survival compared with chemotherapy alone (68% versus 18%; P<0.01) but not the overall survival (68% versus 54%; P=0.48). The presence of FLT3-ITD had a non-significant negative effect on 5-year overall survival compared with non-mutated cases (22% versus 62%; P=0.13). Gene expression profiling showed a unique signature characterized by significantly higher expression of EYA3, SESN1, PRDM2/RIZ, and HIST2H4 genes. In conclusion, t(6; 9)/DEK-NUP214 represents a unique subtype of acute myeloid leukemia with a high risk of relapse, high frequency of FLT3-ITD, and a specific gene expression signature.

U2 - 10.3324/haematol.2013.098517

DO - 10.3324/haematol.2013.098517

M3 - Article

VL - 99

SP - 865

EP - 872

JO - Haematologica-The Hematology Journal

T2 - Haematologica-The Hematology Journal

JF - Haematologica-The Hematology Journal

SN - 1592-8721

IS - 5

ER -