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Childhood near-tetr...
Childhood near-tetraploid acute lymphoblastic leukemia : an EGIL study on 36 cases
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- Lemez, Petr (author)
- 1Department of Hematology and Blood Transfusion, Hospital Jihlava, Jihlava, Czech Republic
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- Attarbaschi, Andishe (author)
- Karolinska Institutet
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- Béné, Marie C (author)
- GEIL-Laboratoire d’Immunologie du CHU & Faculte´ de Me´ decine, Nancy, France
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- Bertrand, Yves (author)
- Department of Pediatric Hematology, Hopital Debrousse,
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- Castoldi, Gianluigi (author)
- Institute of Hematology, University of Ferrara, Ferrara, Italy
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- Forestier, Erik (author)
- Umeå universitet,Pediatrik
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- Garand, Richard (author)
- Laboratory of Hematology, University Hospitals, Nantes, France
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- Haas, Oskar A (author)
- St. Anna Children’s Hospital, Vienna, Austria
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- Kagialis-Girard, Sandrine (author)
- Hematological Laboratory, Hopital Debrousse, Lyon, France
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- Ludwig, Wolf-Dieter (author)
- Department of Hematology-Oncology-Tumor Biology, HELIOS Clinic, Berlin-Buch, Germany
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- Matutes, Estella (author)
- Department of Haematological Oncology, Royal Marsden Hospital, London, UK
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- Mejstríková, Ester (author)
- Department of Pediatric Hematology and Oncology, 2nd Medical School, Charles University and University Hospital Motol, Prague, Czech Republic
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- Pages, Marie-Pierre (author)
- Hematological Laboratory, Hopital Debrousse, Lyon, France
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- Pickl, Winfried (author)
- Institute of Immunology, University of Vienna, Vienna, Austria
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- Porwit, Anna (author)
- Department of Pathology, Karolinska University Hospital and Institute, Stockholm, Sweden
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- Orfao, Alberto (author)
- Servicio di Citometria, University of Salamanca, Salamanca, Spain
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- Schabath, Richard (author)
- Department of Hematology-Oncology-Tumor Biology, HELIOS Clinic, Berlin-Buch, Germany
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- Starý, Jan (author)
- Department of Pediatric Hematology and Oncology, 2nd Medical School, Charles University and University Hospital Motol, Prague, Czech Republic
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- Strobl, Herbert (author)
- Institute of Immunology, University of Vienna, Vienna, Austria
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- Talmant, Pascaline (author)
- Laboratory of Hematology, University Hospitals, Nantes, France
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- van't Veer, Mars B (author)
- Daniel den Hoed Cancer Center, Rotterdam, The Netherlands
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- Zemanová, Zuzana (author)
- Center of Oncocytogenetics, Institute of Clinical Biochemistry and Laboratory Diagnostics, General University Hospital and First Faculty of Medicine, Charles University, Prague, Czech Republic
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(creator_code:org_t)
- 2010-06-19
- 2010
- English.
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In: European Journal of Haematology. - : Wiley. - 0902-4441 .- 1600-0609. ; 85:4, s. 300-308
- Related links:
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https://urn.kb.se/re...
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https://doi.org/10.1...
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Abstract
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- 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.
Keyword
- Tetraploid acute lymphoblastic leukemia; polyploidy; childhood; classification; immunophenotyping; molecular genetics; prognosis
Publication and Content Type
- ref (subject category)
- art (subject category)
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- By the author/editor
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Lemez, Petr
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Attarbaschi, And ...
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Béné, Marie C
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Bertrand, Yves
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Castoldi, Gianlu ...
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Forestier, Erik
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Garand, Richard
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Haas, Oskar A
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Kagialis-Girard, ...
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Ludwig, Wolf-Die ...
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Matutes, Estella
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Mejstríková, Est ...
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Pages, Marie-Pie ...
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Pickl, Winfried
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Porwit, Anna
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Orfao, Alberto
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Schabath, Richar ...
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Starý, Jan
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Strobl, Herbert
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Talmant, Pascali ...
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van't Veer, Mars ...
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Zemanová, Zuzana
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European Journal ...
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Umeå University
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Karolinska Institutet