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).

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.
AuthorsH Hasle, M Aricò, G Basso, A Biondi, A Cantù Rajnoldi, U Creutzig, S Fenu, C Fonatsch, O A Haas, J Harbott, G Kardos, G Kerndrup, G Mann, C M Niemeyer, H Ptoszkova, J Ritter, R Slater, J Starý, B Stollmann-Gibbels, A M Testi, E R van Wering, M Zimmermann
JournalLeukemia (Leukemia) Vol. 13 Issue 3 Pg. 376-85 (Mar 1999) ISSN: 0887-6924 [Print] ENGLAND
PMID10086728 (Publication Type: Journal Article)
  • Acute Disease
  • Child
  • Child, Preschool
  • Chromosomes, Human, Pair 7
  • Female
  • Genetic Predisposition to Disease
  • Humans
  • Infant
  • Leukemia, Myeloid (genetics)
  • Leukemia, Myelomonocytic, Chronic (genetics)
  • Male
  • Monosomy
  • Myelodysplastic Syndromes (genetics)
  • Survival Rate

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.
Realize the full power of the drug-disease research network!

Choose Username:
Verify Password: