PMID- 10086728
OWN - NLM
STAT- MEDLINE
DCOM- 19990407
LR  - 20190915
IS  - 0887-6924 (Print)
IS  - 0887-6924 (Linking)
VI  - 13
IP  - 3
DP  - 1999 Mar
TI  - Myelodysplastic syndrome, juvenile myelomonocytic leukemia, and acute myeloid 
      leukemia associated with complete or partial monosomy 7. European Working Group 
      on MDS in Childhood (EWOG-MDS).
PG  - 376-85
AB  - We reviewed the clinical features, treatment, and outcome of 100 children with 
      myelodysplastic syndrome (MDS), juvenile myelomonocytic leukemia (JMML), and 
      acute myeloid leukemia (AML) associated with complete monosomy 7 (-7) or deletion 
      of the long arm of chromosome 7 (7q-). Patients with therapy-induced disease were 
      excluded. The morphologic diagnoses according to modified FAB criteria were: MDS 
      in 72 (refractory anemia (RA) in 11, RA with excess of blasts (RAEB) in eight, 
      RAEB in transformation (RAEB-T) in 10, JMML in 43), and AML in 28. The median age 
      at presentation was 2.8 years (range 2 months to 15 years), being lowest in JMML 
      (1.1 year). Loss of chromosome 7 as the sole cytogenetic abnormality was observed 
      in 75% of those with MDS compared with 32% of those with AML. Predisposing 
      conditions (including familial MDS/AML) were found in 20%. Three-year survival 
      was 82% in RA, 63% in RAEB, 45% in JMML, 34% in AML, and 8% in RAEB-T. Children 
      with -7 alone had a superior survival than those with other cytogenetic 
      abnormalities: this was solely due to a better survival in MDS (3-year survival 
      56 vs 24%). The reverse was found in AML (3-year survival 13% in -7 alone vs 44% 
      in other cytogenetic groups). Stable disease for several years was documented in 
      more than half the patients with RA or RAEB. Patients with RA, RAEB or JMML 
      treated with bone marrow transplantation (BMT) without prior chemotherapy had a 
      3-year survival of 73%. The morphologic diagnosis was the strongest prognostic 
      factor. Only patients with a diagnosis of JMML fitted what has previously been 
      referred to as the monosomy 7 syndrome. Our data give no support to the concept 
      of monosomy 7 as a distinct syndrome.
FAU - Hasle, H
AU  - Hasle H
AD  - Department of Pediatrics, Aarhus University Hospital, Denmark.
FAU - Arico, M
AU  - Arico M
FAU - Basso, G
AU  - Basso G
FAU - Biondi, A
AU  - Biondi A
FAU - Cantu Rajnoldi, A
AU  - Cantu Rajnoldi A
FAU - Creutzig, U
AU  - Creutzig U
FAU - Fenu, S
AU  - Fenu S
FAU - Fonatsch, C
AU  - Fonatsch C
FAU - Haas, O A
AU  - Haas OA
FAU - Harbott, J
AU  - Harbott J
FAU - Kardos, G
AU  - Kardos G
FAU - Kerndrup, G
AU  - Kerndrup G
FAU - Mann, G
AU  - Mann G
FAU - Niemeyer, C M
AU  - Niemeyer CM
FAU - Ptoszkova, H
AU  - Ptoszkova H
FAU - Ritter, J
AU  - Ritter J
FAU - Slater, R
AU  - Slater R
FAU - Stary, J
AU  - Stary J
FAU - Stollmann-Gibbels, B
AU  - Stollmann-Gibbels B
FAU - Testi, A M
AU  - Testi AM
FAU - van Wering, E R
AU  - van Wering ER
FAU - Zimmermann, M
AU  - Zimmermann M
LA  - eng
PT  - Journal Article
PL  - England
TA  - Leukemia
JT  - Leukemia
JID - 8704895
SB  - IM
MH  - Acute Disease
MH  - Child
MH  - Child, Preschool
MH  - *Chromosomes, Human, Pair 7
MH  - Female
MH  - Genetic Predisposition to Disease
MH  - Humans
MH  - Infant
MH  - Leukemia, Myeloid/*genetics
MH  - Leukemia, Myelomonocytic, Chronic/*genetics
MH  - Male
MH  - *Monosomy
MH  - Myelodysplastic Syndromes/*genetics
MH  - Survival Rate
EDAT- 1999/03/23 00:00
MHDA- 1999/03/23 00:01
CRDT- 1999/03/23 00:00
PHST- 1999/03/23 00:00 [pubmed]
PHST- 1999/03/23 00:01 [medline]
PHST- 1999/03/23 00:00 [entrez]
AID - 10.1038/sj.leu.2401342 [doi]
PST - ppublish
SO  - Leukemia. 1999 Mar;13(3):376-85. doi: 10.1038/sj.leu.2401342.