title: The International Prognostic Scoring System (IPSS) for childhood myelodysplastic syndrome (MDS) and juvenile myelomonocytic leukemia (JMML).
abstract: The International Prognostic Scoring System (IPSS) for myelodysplastic syndrome (MDS) is based upon weighted data on bone marrow (BM) blast percentage, cytopenia, and cytogenetics, separating patients into four prognostic groups. We analyzed the value of the IPSS in 142 children with de novo MDS and 166 children with juvenile myelomonocytic leukemia (JMML) enrolled in retro- and prospective studies of the European Working Group on childhood MDS (EWOG-MDS). Survivals in MDS and JMML were analyzed separately. Among the criteria considered by the IPSS score, only BM blasts <5% and platelets >100 x 10(9)/l were significantly associated with a superior survival in MDS. In JMML, better survival was associated with platelets >40 x 10(9)/l, but not with any other IPSS factors including cytogenetics. In conclusion, the IPSS is of limited value in both pediatric MDS and JMML. The results reflect the differences between myelodysplastic and myeloproliferative diseases in children and adults.
authors.affiliation: Department of Pediatrics, Skejby Hospital, Aarhus University, Denmark. hasle@dadlnet.dk
authors.forename: H
authors.initials: H
authors.lastname: Hasle
authors.forename: I
authors.initials: I
authors.lastname: Baumann
authors.forename: E
authors.initials: E
authors.lastname: Bergsträsser
authors.forename: S
authors.initials: S
authors.lastname: Fenu
authors.forename: A
authors.initials: A
authors.lastname: Fischer
authors.forename: G
authors.initials: G
authors.lastname: Kardos
authors.forename: G
authors.initials: G
authors.lastname: Kerndrup
authors.forename: F
authors.initials: F
authors.lastname: Locatelli
authors.forename: T
authors.initials: T
authors.lastname: Rogge
authors.forename: K R
authors.initials: KR
authors.lastname: Schultz
authors.forename: J
authors.initials: J
authors.lastname: Starý
authors.forename: M
authors.initials: M
authors.lastname: Trebo
authors.forename: M M
authors.initials: MM
authors.lastname: van den Heuvel-Eibrink
authors.forename: J
authors.initials: J
authors.lastname: Harbott
authors.forename: P
authors.initials: P
authors.lastname: Nöllke
authors.forename: C M
authors.initials: CM
authors.lastname: Niemeyer
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D015479:Leukemia, Myelomonocytic, Acute / Q000175:diagnosis*; D015477:Leukemia, Myelomonocytic, Chronic / Q000175:diagnosis*; D008297:Male; D009190:Myelodysplastic Syndromes / Q000175:diagnosis*; D009367:Neoplasm Staging; D011379:Prognosis; D011446:Prospective Studies; D012189:Retrospective Studies; D015996:Survival Rate