Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
SHR Neuro Krebs Kardio Lipid Stoffw Microb
Lemez, P; Attarbaschi, A; Béné, MC; Bertrand, Y; Castoldi, G; Forestier, E; Garand, R; Haas, OA; Kagialis-Girard, S; Ludwig, WD; Matutes, E; Mejstríková, E; Pages, MP; Pickl, W; Porwit, A; Orfao, A; Schabath, R; Starı, J; Strobl, H; Talmant, P; van't Veer, MB; Zemanová, Z; European Group for the Immunological Characterization of Leukemias (EGIL).
Childhood near-tetraploid acute lymphoblastic leukemia: an EGIL study on 36 cases.
Eur J Haematol. 2010; 85(4):300-308
Doi: 10.1111/j.1600-0609.2010.01493.x
Web of Science
PubMed
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- Co-Autor*innen der Med Uni Graz
- Strobl Herbert
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- Abstract:
- Objectives: Patients with near-tetraploid (karyotype: 81 - 103 chromosomes) acute lymphoblastic leukemia (NT-ALL) constitute about 1% of childhood ALL and data reported on them are limited and controversial. The aim of the study was to enlarge the knowledge on these rarely occurring ALL. Methods: The members of the European Group for Immunophenotyping of Leukemias (EGIL) searched retrospectively their databases for NT-ALL patients. Results: We collected data of 36 European children from seven European countries with NT-ALL diagnosed since 1992. All patients reached complete remission (CR) after induction chemotherapy. Their blasts were negative for peroxidase and BCR-ABL1. Ten children were diagnosed as T-cell ALL (T-ALL) EGIL categories (T-I n = 2, T-II n = 2, T-III n = 3, T-IV n = 3) and four displayed various structural chromosomal abnormalities. Eight of 10 T-ALL remained in 1st CR; one died in CR from sepsis and one is alive in 2nd CR. Median survival was 88 (7-213) months. B-cell precursor (BCP) ALL was diagnosed in 26 children. Thirteen were positive for ETV6-RUNX1 and are alive in 1st CR for 32-147 months. Ten children were ETV6-RUNX1 negative and remained in 1st CR for 16-163 months. One girl with hypodiploid and NT metaphases and ETV6-RUNX1-negative BCP-ALL and one of two boys with NT-BCP-ALL not examined for ETV6-RUNX1 died of infection after stem cell transplantation in 2nd/3rd CR. Secondary myelodysplastic syndrome developed in two patients with NT-BCP-ALL. Conclusions: Our data demonstrate immunophenotypic, cytogenetic, and molecular heterogeneity of NT-ALL and favorable prognosis of most NT-ALL across different immunophenotypic and/or genetic ALL subtypes.
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- Find related publications in this database (Keywords)
- Tetraploid acute lymphoblastic leukemia
- polyploidy
- childhood
- classification
- immunophenotyping
- molecular genetics
- prognosis