The International Prognostic Scoring System (IPSS) for childhood myelodysplastic syndrome (MDS) and juvenile myelomonocytic leukemia (JMML)

• Published in: Leukemia Vol. 18; no. 12; pp. 2008 - 2014
• Main Authors: HASLE, H, BAUMANN, I, STARY, J, TREBO, M, VAN DEN HEUVEL-EIBRINK, M. M, HARBOTT, J, NÖLLKE, P, NIEMEYER, C. M, BERGSTRÄSSER, E, FENU, S, FISCHER, A, KARDOS, G, KERNDRUP, G, LOCATELLI, F, ROGGE, T, SCHULTZ, K. R
• Format: Journal Article
• Language: English
• Published: London Nature Publishing 01-12-2004; Nature Publishing Group
• Subjects: Anemia; Biological and medical sciences; Bone marrow; Child; Child, Preschool; Children; Congenital diseases; Cytogenetics; Female; Hematologic and hematopoietic diseases; Hematology; Hemoglobin; Hospitals; Humans; Juveniles; Leukemia; Leukemia, Myelomonocytic, Acute - diagnosis; Leukemia, Myelomonocytic, Chronic - diagnosis; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Male; Medical prognosis; Medical sciences; Myelodysplastic syndrome; Myelodysplastic syndromes; Myelodysplastic Syndromes - diagnosis; Myelomonocytic leukemia; Myeloproliferative diseases; Neoplasm Staging; Oncology; Pediatrics; Platelets; Prognosis; Prospective Studies; Retrospective Studies; Survival; Survival Rate; Working groups; Denmark; Italy; Germany; Aarhus Denmark; Human; JMML; Myelomonocytic leukemia; Prognosis; Hematology; International system; Evaluation scale; IPSS; Malignant hemopathy; Myelodysplastic syndrome; children; Child; MDS
• ISSN: 0887-6924; 1476-5551
• DOI: 10.1038/sj.leu.2403489

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.