PMID- 20561032
OWN - NLM
STAT- MEDLINE
DCOM- 20101004
LR  - 20131121
IS  - 1600-0609 (Electronic)
IS  - 0902-4441 (Linking)
VI  - 85
IP  - 4
DP  - 2010 Oct
TI  - Childhood near-tetraploid acute lymphoblastic leukemia: an EGIL study on 36 
      cases.
PG  - 300-8
LID - 10.1111/j.1600-0609.2010.01493.x [doi]
AB  - 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.
CI  - (c) 2010 John Wiley & Sons A/S.
FAU - Lemez, Petr
AU  - Lemez P
AD  - Department of Hematology and Blood Transfusion, Hospital Jihlava, Jihlava, Czech 
      Republic. plemez@post.cz
FAU - Attarbaschi, Andishe
AU  - Attarbaschi A
FAU - Bene, Marie C
AU  - Bene MC
FAU - Bertrand, Yves
AU  - Bertrand Y
FAU - Castoldi, Gianluigi
AU  - Castoldi G
FAU - Forestier, Erik
AU  - Forestier E
FAU - Garand, Richard
AU  - Garand R
FAU - Haas, Oskar A
AU  - Haas OA
FAU - Kagialis-Girard, Sandrine
AU  - Kagialis-Girard S
FAU - Ludwig, Wolf-Dieter
AU  - Ludwig WD
FAU - Matutes, Estella
AU  - Matutes E
FAU - Mejstrikova, Ester
AU  - Mejstrikova E
FAU - Pages, Marie-Pierre
AU  - Pages MP
FAU - Pickl, Winfried
AU  - Pickl W
FAU - Porwit, Anna
AU  - Porwit A
FAU - Orfao, Alberto
AU  - Orfao A
FAU - Schabath, Richard
AU  - Schabath R
FAU - Stary, Jan
AU  - Stary J
FAU - Strobl, Herbert
AU  - Strobl H
FAU - Talmant, Pascaline
AU  - Talmant P
FAU - van't Veer, Mars B
AU  - van't Veer MB
FAU - Zemanova, Zuzana
AU  - Zemanova Z
CN  - European Group for the Immunological Characterization of Leukemias (EGIL)
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - England
TA  - Eur J Haematol
JT  - European journal of haematology
JID - 8703985
RN  - 0 (Core Binding Factor Alpha 2 Subunit)
RN  - 0 (Oncogene Proteins, Fusion)
RN  - 0 (TEL-AML1 fusion protein)
RN  - EC 2.7.10.2 (Fusion Proteins, bcr-abl)
SB  - IM
MH  - Antineoplastic Combined Chemotherapy Protocols/therapeutic use
MH  - Child
MH  - Chromosome Aberrations
MH  - Core Binding Factor Alpha 2 Subunit
MH  - Europe
MH  - Female
MH  - Fusion Proteins, bcr-abl/genetics
MH  - Humans
MH  - Immunophenotyping
MH  - Karyotyping
MH  - Male
MH  - Oncogene Proteins, Fusion/genetics
MH  - *Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug 
      therapy/epidemiology/genetics/immunology/mortality
MH  - Prognosis
MH  - Remission Induction
MH  - Retrospective Studies
MH  - Survival Analysis
MH  - Treatment Outcome
EDAT- 2010/06/22 06:00
MHDA- 2010/10/05 06:00
CRDT- 2010/06/22 06:00
PHST- 2010/06/22 06:00 [entrez]
PHST- 2010/06/22 06:00 [pubmed]
PHST- 2010/10/05 06:00 [medline]
AID - EJH1493 [pii]
AID - 10.1111/j.1600-0609.2010.01493.x [doi]
PST - ppublish
SO  - Eur J Haematol. 2010 Oct;85(4):300-8. doi: 10.1111/j.1600-0609.2010.01493.x.