Monosomy 7 (-7) and deletion 7q [del(7q)] are rare in childhood acute myeloid leukemia (AML). We retrospectively collected data on 258 children with AML or refractory anemia with excess blasts in transformation (RAEB-T) and -7 or del(7q) with or without other cytogenetic aberrations [+/- other]. Karyotypes included -7 (n = 90), -7 other (n = 82), del(7q) (n = 21), and del(7q) other(n = 65). Complete remission (CR) was achieved in fewer patients with -7 +/- other compared with del(7q) +/- other (61% versus 89%, P <.001). Overall, the 5-year survival rate was 39% (SE, 3%). Survival was superior in del(7q) +/- other compared with -7 other (51 % versus 30%, P <.01). Cytogenetic aberrations considered favorable in AML [t(8;21)(q22;q22), inv(16)(p13q22), t(15;17)(q22;q21), t(9;11)(p22;q23)] (n = 24) were strongly associated with del(7q) and a higher 5-year survival rate compared with del(7q) without favorable cytogenetics (75% versus 46%, P =.03). Patients with -7 and inv(3),-5/del(5q), or +21 had a 5-year survival rate of 5%. Stem cell transplantation analyzed as a time-dependent variable had no impact on overall survival. However, patients not achieving CR had a 31% survival rate after stem cell transplantation. Childhood AML with chromosome 7 aberrations represents a heterogeneous group of disorders with additional cytogenetic aberrations having a major prognostic impact which should be reflected in future risk-group stratification.
Hasle, H., Alonzo, T. A., Auvrignon, A., Behar, C., Chang, M., Creutzig, U., Fischer, A., Forestier, E., Fynn, A., Haas, O. A., Harbott, J., Harrison, C. J., Heerema, N. A., Van Den Heuvel-Eibrink, M. M., Kaspers, G. J. L., Locatelli, F., Noellke, P., Polychronopoulou, S., Ravindranath, Y., Razzouk, B., Reinhardt, D., Savva, N. N., Stark, B., Suciu, S., Tsukimoto, I., Webb, D. K., Wojcik, D., Woods, W. G., Zimmermann, M., Niemeyer, C. M., Raimondi, S. C., Monosomy 7 and deletion 7q in children and adolescents with acute myeloid leukemia: an international retrospective study, <<BLOOD>>, 2007; 109 (11): 4641-4647. [doi:10.1182/blood-2006-10-051342] [https://hdl.handle.net/10807/257399]
Monosomy 7 and deletion 7q in children and adolescents with acute myeloid leukemia: an international retrospective study
Locatelli, Franco;
2007
Abstract
Monosomy 7 (-7) and deletion 7q [del(7q)] are rare in childhood acute myeloid leukemia (AML). We retrospectively collected data on 258 children with AML or refractory anemia with excess blasts in transformation (RAEB-T) and -7 or del(7q) with or without other cytogenetic aberrations [+/- other]. Karyotypes included -7 (n = 90), -7 other (n = 82), del(7q) (n = 21), and del(7q) other(n = 65). Complete remission (CR) was achieved in fewer patients with -7 +/- other compared with del(7q) +/- other (61% versus 89%, P <.001). Overall, the 5-year survival rate was 39% (SE, 3%). Survival was superior in del(7q) +/- other compared with -7 other (51 % versus 30%, P <.01). Cytogenetic aberrations considered favorable in AML [t(8;21)(q22;q22), inv(16)(p13q22), t(15;17)(q22;q21), t(9;11)(p22;q23)] (n = 24) were strongly associated with del(7q) and a higher 5-year survival rate compared with del(7q) without favorable cytogenetics (75% versus 46%, P =.03). Patients with -7 and inv(3),-5/del(5q), or +21 had a 5-year survival rate of 5%. Stem cell transplantation analyzed as a time-dependent variable had no impact on overall survival. However, patients not achieving CR had a 31% survival rate after stem cell transplantation. Childhood AML with chromosome 7 aberrations represents a heterogeneous group of disorders with additional cytogenetic aberrations having a major prognostic impact which should be reflected in future risk-group stratification.
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