Comprehensive analysis of isolated der(1;7)(q10;p10) in a large international homogenous cohort of patients with myelodysplastic syndromes
Standard
Comprehensive analysis of isolated der(1;7)(q10;p10) in a large international homogenous cohort of patients with myelodysplastic syndromes. / Ganster, Christina; Müller-Thomas, Catharina; Haferlach, Claudia; Strupp, Corinna; Ogata, Kiyoyuki; Germing, Ulrich; Hildebrandt, Barbara; Mallo, Mar; Lübbert, Michael; Müller, Christel; Solé, Francesc; Götze, Katharina S; Vandenberghe, Peter; Göhring, Gudrun; Steinmetz, Tilman; Kröger, Nicolaus; Platzbecker, Uwe; Söling, Ulrike; Raynaud, Sophie; Shirneshan, Katayoon; Schanz, Julie; Haase, Detlef.
in: GENE CHROMOSOME CANC, Jahrgang 58, Nr. 10, 10.2019, S. 689-697.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
Harvard
APA
Vancouver
Bibtex
}
RIS
TY - JOUR
T1 - Comprehensive analysis of isolated der(1;7)(q10;p10) in a large international homogenous cohort of patients with myelodysplastic syndromes
AU - Ganster, Christina
AU - Müller-Thomas, Catharina
AU - Haferlach, Claudia
AU - Strupp, Corinna
AU - Ogata, Kiyoyuki
AU - Germing, Ulrich
AU - Hildebrandt, Barbara
AU - Mallo, Mar
AU - Lübbert, Michael
AU - Müller, Christel
AU - Solé, Francesc
AU - Götze, Katharina S
AU - Vandenberghe, Peter
AU - Göhring, Gudrun
AU - Steinmetz, Tilman
AU - Kröger, Nicolaus
AU - Platzbecker, Uwe
AU - Söling, Ulrike
AU - Raynaud, Sophie
AU - Shirneshan, Katayoon
AU - Schanz, Julie
AU - Haase, Detlef
N1 - © 2019 Wiley Periodicals, Inc.
PY - 2019/10
Y1 - 2019/10
N2 - The karyotype is a strong independent prognostic factor in myelodysplastic syndromes (MDS). Since the implementation of the new comprehensive cytogenetic scoring system for MDS, chromosome 7 anomalies are no longer generally assigned to poor risk features but are thoroughly separated. However, der(1;7)(q10;p10), hereinafter der(1;7), is merged into the group labeled "any other single" and belongs to the intermediate risk group, just by definition due to lack of adequate clinical data. The aim of our international collaborative was to clarify the "real" prognostic impact of der(1;7) on a homogenous and well-documented data base. We performed detailed analysis of 63 MDS patients with isolated der(1;7) constituting the largest cohort hitherto reported. Furthermore, clinical data are compared with those of patients with isolated del(7q) and isolated monosomy 7. Median overall survival (OS) of patients with der(1;7) is 26 months (hazard ratio (HR) 0.91 for del(7q) vs der(1;7) and 2.53 for monosomy 7 vs der(1;7)). The der(1;7) is associated with profound thrombocytopenia most probably causing the reduced OS which is in striking contrast to the low risk for AML transformation (HR 3.89 for del(7q) vs der(1;7) and 5.88 for monosomy 7 vs der(1;7)). Molecular karyotyping indicates that der(1;7) is generated in a single step during mitosis and that a chromosomal imbalance rather than a single disrupted gene accounts for malignancy. Thus, the current cytogenetic scoring system assigning isolated der(1;7) to the intermediate risk group is now confirmed by a sufficient data set.
AB - The karyotype is a strong independent prognostic factor in myelodysplastic syndromes (MDS). Since the implementation of the new comprehensive cytogenetic scoring system for MDS, chromosome 7 anomalies are no longer generally assigned to poor risk features but are thoroughly separated. However, der(1;7)(q10;p10), hereinafter der(1;7), is merged into the group labeled "any other single" and belongs to the intermediate risk group, just by definition due to lack of adequate clinical data. The aim of our international collaborative was to clarify the "real" prognostic impact of der(1;7) on a homogenous and well-documented data base. We performed detailed analysis of 63 MDS patients with isolated der(1;7) constituting the largest cohort hitherto reported. Furthermore, clinical data are compared with those of patients with isolated del(7q) and isolated monosomy 7. Median overall survival (OS) of patients with der(1;7) is 26 months (hazard ratio (HR) 0.91 for del(7q) vs der(1;7) and 2.53 for monosomy 7 vs der(1;7)). The der(1;7) is associated with profound thrombocytopenia most probably causing the reduced OS which is in striking contrast to the low risk for AML transformation (HR 3.89 for del(7q) vs der(1;7) and 5.88 for monosomy 7 vs der(1;7)). Molecular karyotyping indicates that der(1;7) is generated in a single step during mitosis and that a chromosomal imbalance rather than a single disrupted gene accounts for malignancy. Thus, the current cytogenetic scoring system assigning isolated der(1;7) to the intermediate risk group is now confirmed by a sufficient data set.
U2 - 10.1002/gcc.22760
DO - 10.1002/gcc.22760
M3 - SCORING: Journal article
C2 - 30994215
VL - 58
SP - 689
EP - 697
JO - GENE CHROMOSOME CANC
JF - GENE CHROMOSOME CANC
SN - 1045-2257
IS - 10
ER -