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Pediatric myelodysplasia: a study of 68 children and a new prognostic
scoring system
SJ Passmore, IM Hann, CA Stiller, P Ramani, GJ Swansbury, B Gibbons, BR Reeves and JM Chessells
Institute of Child Health, London, UK.
Clinical, morphologic, and cytogenetic features were examined in a group of
68 children with myelodysplasia (MDS) referred to a single institution
between 1971-1991. The morphologic French-American-British (FAB) system of
classification proved of limited value in this group of patients because
50% of the cases were categorized as chronic myelomonocytic leukemia and
three patients with eosinophilia and MDS were unclassifiable. Cytogenetic
analysis was performed in 63 cases and clonal abnormalities were detected
in 55%; the most common chromosome involved was number 7. Modification of
the FAB system to incorporate additional diagnostic features such as
pretreatment fetal hemoglobin (Hb F) and cytogenetics allowed incorporation
of the categories of juvenile chronic myeloid leukemia (JCML) and infantile
monosomy 7 syndrome (IMo7). The resulting groups of patients had highly
significant differences in survival (P = .00009). The overall 5-year
survival for the patients was 31.9% (95% CI 21.7 to 44.1) and factors
influencing prognosis included: modified FAB type, platelet count, Hb F
level, and cytogenetic complexity. We developed a scoring system ("FPC")
where each of the following findings at diagnosis scored one point: HbF
greater than 10%, platelets < or = 40 x 10(9)/L, and complex karyotypic
changes (two or more clonal structural/numerical abnormalities), which
produced groups with highly significant differences, patients with a score
of 0 having a 5-year survival of 61.6% (CI 33% to 84%), whereas those with
a score of two or three all died within 4 years of diagnosis. The revised
classification and scoring system may prove helpful in making treatment
choices in pediatric MDS and now needs to be tested prospectively in large
scale population-based studies.
Volume 85,
Issue 7,
pp. 1742-1750,
04/01/1995
Copyright © 1995 by The American Society of Hematology
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