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  • Lemez, Petr1Department of Hematology and Blood Transfusion, Hospital Jihlava, Jihlava, Czech Republic (author)

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

  • Article/chapterEnglish2010

Publisher, publication year, extent ...

  • 2010-06-19
  • Wiley,2010
  • printrdacarrier

Numbers

  • LIBRIS-ID:oai:DiVA.org:umu-36703
  • https://urn.kb.se/resolve?urn=urn:nbn:se:umu:diva-36703URI
  • https://doi.org/10.1111/j.1600-0609.2010.01493.xDOI
  • http://kipublications.ki.se/Default.aspx?queryparsed=id:121293179URI

Supplementary language notes

  • Language:English
  • Summary in:English

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  • Subject category:ref swepub-contenttype
  • Subject category:art swepub-publicationtype

Notes

  • 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.

Subject headings and genre

  • Tetraploid acute lymphoblastic leukemia; polyploidy; childhood; classification; immunophenotyping; molecular genetics; prognosis

Added entries (persons, corporate bodies, meetings, titles ...)

  • Attarbaschi, AndisheKarolinska Institutet (author)
  • Béné, Marie CGEIL-Laboratoire d’Immunologie du CHU & Faculte´ de Me´ decine, Nancy, France (author)
  • Bertrand, YvesDepartment of Pediatric Hematology, Hopital Debrousse, (author)
  • Castoldi, GianluigiInstitute of Hematology, University of Ferrara, Ferrara, Italy (author)
  • Forestier, ErikUmeå universitet,Pediatrik(Swepub:umu)erfo0007 (author)
  • Garand, RichardLaboratory of Hematology, University Hospitals, Nantes, France (author)
  • Haas, Oskar ASt. Anna Children’s Hospital, Vienna, Austria (author)
  • Kagialis-Girard, SandrineHematological Laboratory, Hopital Debrousse, Lyon, France (author)
  • Ludwig, Wolf-DieterDepartment of Hematology-Oncology-Tumor Biology, HELIOS Clinic, Berlin-Buch, Germany (author)
  • Matutes, EstellaDepartment of Haematological Oncology, Royal Marsden Hospital, London, UK (author)
  • Mejstríková, EsterDepartment of Pediatric Hematology and Oncology, 2nd Medical School, Charles University and University Hospital Motol, Prague, Czech Republic (author)
  • Pages, Marie-PierreHematological Laboratory, Hopital Debrousse, Lyon, France (author)
  • Pickl, WinfriedInstitute of Immunology, University of Vienna, Vienna, Austria (author)
  • Porwit, AnnaDepartment of Pathology, Karolinska University Hospital and Institute, Stockholm, Sweden (author)
  • Orfao, AlbertoServicio di Citometria, University of Salamanca, Salamanca, Spain (author)
  • Schabath, RichardDepartment of Hematology-Oncology-Tumor Biology, HELIOS Clinic, Berlin-Buch, Germany (author)
  • Starý, JanDepartment of Pediatric Hematology and Oncology, 2nd Medical School, Charles University and University Hospital Motol, Prague, Czech Republic (author)
  • Strobl, HerbertInstitute of Immunology, University of Vienna, Vienna, Austria (author)
  • Talmant, PascalineLaboratory of Hematology, University Hospitals, Nantes, France (author)
  • van't Veer, Mars BDaniel den Hoed Cancer Center, Rotterdam, The Netherlands (author)
  • Zemanová, ZuzanaCenter of Oncocytogenetics, Institute of Clinical Biochemistry and Laboratory Diagnostics, General University Hospital and First Faculty of Medicine, Charles University, Prague, Czech Republic (author)
  • Karolinska Institutet1Department of Hematology and Blood Transfusion, Hospital Jihlava, Jihlava, Czech Republic (creator_code:org_t)

Related titles

  • In:European Journal of Haematology: Wiley85:4, s. 300-3080902-44411600-0609

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