Childhood near-tetraploid acute lymphoblastic leukemia: an EGIL study on 36 cases

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Childhood near-tetraploid acute lymphoblastic leukemia : an EGIL study on 36 cases

Lemez, Petr (författare): 1Department of Hematology and Blood Transfusion, Hospital Jihlava, Jihlava, Czech Republic

Attarbaschi, Andishe (författare): Karolinska Institutet

Béné, Marie C (författare): GEIL-Laboratoire d’Immunologie du CHU & Faculte´ de Me´ decine, Nancy, France

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Bertrand, Yves (författare): Department of Pediatric Hematology, Hopital Debrousse,

Castoldi, Gianluigi (författare): Institute of Hematology, University of Ferrara, Ferrara, Italy

Forestier, Erik (författare): Umeå universitet,Pediatrik

Garand, Richard (författare): Laboratory of Hematology, University Hospitals, Nantes, France

Haas, Oskar A (författare): St. Anna Children’s Hospital, Vienna, Austria

Kagialis-Girard, Sandrine (författare): Hematological Laboratory, Hopital Debrousse, Lyon, France

Ludwig, Wolf-Dieter (författare): Department of Hematology-Oncology-Tumor Biology, HELIOS Clinic, Berlin-Buch, Germany

Matutes, Estella (författare): Department of Haematological Oncology, Royal Marsden Hospital, London, UK

Mejstríková, Ester (författare): Department of Pediatric Hematology and Oncology, 2nd Medical School, Charles University and University Hospital Motol, Prague, Czech Republic

Pages, Marie-Pierre (författare): Hematological Laboratory, Hopital Debrousse, Lyon, France

Pickl, Winfried (författare): Institute of Immunology, University of Vienna, Vienna, Austria

Porwit, Anna (författare): Department of Pathology, Karolinska University Hospital and Institute, Stockholm, Sweden

Orfao, Alberto (författare): Servicio di Citometria, University of Salamanca, Salamanca, Spain

Schabath, Richard (författare): Department of Hematology-Oncology-Tumor Biology, HELIOS Clinic, Berlin-Buch, Germany

Starý, Jan (författare): Department of Pediatric Hematology and Oncology, 2nd Medical School, Charles University and University Hospital Motol, Prague, Czech Republic

Strobl, Herbert (författare): Institute of Immunology, University of Vienna, Vienna, Austria

Talmant, Pascaline (författare): Laboratory of Hematology, University Hospitals, Nantes, France

van't Veer, Mars B (författare): Daniel den Hoed Cancer Center, Rotterdam, The Netherlands

Zemanová, Zuzana (författare): 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
Engelska.
Ingår i: European Journal of Haematology. - : Wiley. - 0902-4441 .- 1600-0609. ; 85:4, s. 300-308

Relaterad länk:: https://urn.kb.se/re...; visa fler...; https://doi.org/10.1...; http://kipublication...; visa färre...

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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.

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Childhood near-tetraploid acute lymphoblastic leukemia : an EGIL study on 36 cases

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