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Childhood near-tetraploid acute lymphoblastic leukemia: an EGIL study on 36 cases
1Department of Hematology and Blood Transfusion, Hospital Jihlava, Jihlava, Czech Republic.
St. Anna Children’s Hospital, Vienna, Austria.
GEIL-Laboratoire d’Immunologie du CHU & Faculte´ de Me´ decine, Nancy, France.
Department of Pediatric Hematology, Hopital Debrousse,.
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2010 (Engelska)Ingår i: European Journal of Haematology, ISSN 0902-4441, E-ISSN 1600-0609, Vol. 85, nr 4, s. 300-308Artikel i tidskrift (Refereegranskat) Published
Abstract [en]

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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2010. Vol. 85, nr 4, s. 300-308
Nyckelord [en]
Tetraploid acute lymphoblastic leukemia; polyploidy; childhood; classification; immunophenotyping; molecular genetics; prognosis
Identifikatorer
URN: urn:nbn:se:umu:diva-36703DOI: 10.1111/j.1600-0609.2010.01493.xISI: 000282049300003PubMedID: 20561032OAI: oai:DiVA.org:umu-36703DiVA, id: diva2:355810
Tillgänglig från: 2010-10-08 Skapad: 2010-10-08 Senast uppdaterad: 2018-06-08Bibliografiskt granskad

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