Konference: 2007 49th ASH Annual Meeting - účast ČR
Kategorie:
Nádory dětského a adolescentního věku
Téma: Postery
Číslo abstraktu: 2452
Autoři: Gohrin Gudrun; prof. Ing. Kyra Michalová, DrSc.; H.Berna Beverloo, PhD; MD David Betts; Jochen Harbott; Prof. Dr. Oskar A. Haas; Gitte Kerndrup; Laura Sainati; Eva Bergstraesser; Prof. MD Henrik Hasle; prof. MUDr. Jan Starý, DrSc.; Monika Trebo; MD Marry M. van den Heuvel-Eibrink, PhD; MD Marco Zecca; Alexandra Fischer, R.N.; Peter Noellke
To study the clinical significance of recurrent chromosome
aberrations in childhood MDS, cytogenetic data of 394 consecutive
children with refractory cytopenia (RC) (N=215), RAEB (N=141) and
RAEB-T (N=38) analyzed in the regional cytogenetic reference
centers and registered in the prospective study EWOG-MDS 98 between
1998 and 2005 were evaluated. At diagnosis, a karyotype could be
defined in 279/394 patients (pts) (71%). No karyotype was obtained
in 16% of pts with RC compared to 8% pts with RAEB and RAEB-t
(p<0.001). Clonal chromosome aberrations were more common in pts
with advanced MDS (RAEB and RAEB-T, 61%) compared to RC (29%), and
in pts with secondary (69%) compared to primary MDS (36%)
(p<0.001). Monosomy 7 was the most frequent aberration occurring
with similar frequency in RC (47% of abnormal karyotypes) compared
to advanced MDS (49%) and in primary (53%) compared to secondary
(41%) MDS. In addition, aberrations typical for de novo AML such as
aberrations involving 11q23 or 3q, t(6;9) and del(9q) were noted in
morphologically and clinically unequivocal MDS cases. Recurrent
aberrations of adult MDS like isolated del(5q), del(20q) and -Y
were very uncommon indicating a different pathogenesis of these
cases. In pts with advanced MDS, there was no significant
difference in overall survival (OS) of pts with normal karyotype
(44% 18) compared to pts with monosomy 7 (58% 19) and patients with
other karyotypes (61% 22). However, pts with advanced MDS and a
complex karyotype (defined by 3 chromosome aberrations, presence of
structural aberrations and excluding clonal evolution of monosomy
7) had a shorter OS (16% 15, p<0.01). OS and event-free survival
after hematopoietic stem cell transplantation (HSCT) in pts with
complex karyotypes was inferior compared to that of pts with other
cytogenetic aberrations (p=0.012 and 0.039, respectively). Within
the group of pts with secondary MDS, complex karyotypes were found
in MDS evolving from inherited bone marrow failure disorders or
after radio-/ chemotherapy, but absent in familial MDS and cases
evolving from acquired aplastic anemia. As shown in a multivariate
Cox analysis, advanced MDS, secondary MDS, the presence of a
complex karyotype and HSCT were identified as independent
prognostic factors for OS. Thus, this study demonstrates the
prognostic significance of cytogenetic findings in advanced
childhood MDS independent of HSCT.
Abstract #2452 appears in Blood, Volume 110, issue 11, November 16,
2007
Keywords: Complex Aberrant Karyotype|MDS|Childhood
Disclosure: No relevant conflicts of interest to declare.
Sunday, December 9, 2007 6:00 PM
Session Info: Poster Session: Myelodysplastic Syndromes: Prognosis
and Clinical Correlative Studies (6:00 p.m.-8:00 p.m.)
Datum přednesení příspěvku: 9. 12. 2007
