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Revista chilena de pediatría

versión impresa ISSN 0370-4106

Resumen

OYARCE L, VERÓNICA  ; RODRIGUEZ Z, NATALIE  ; TORDECILLA C, JUAN    y  VERDUGO L, PATRICIA  . Myelodysplasia in Pediatric Patients: Evaluation of Current Classifications. Rev. chil. pediatr. [online]. 2009, vol.80, n.4, pp.339-346. ISSN 0370-4106.  http://dx.doi.org/10.4067/S0370-41062009000400005.

Introduction: Myelodysplastic Syndrome (MDS) in pediatrics is a rare and difficult clinical picture to diagnose. Classifications currently in use do not always help in identifying risk factors for leukemic transformation in children. Objective: To evaluate the value of current classifications by simultaneously determining a) the frequency of MDS in a pediatric population b) applying CCC classification (category, cytopenias, cytogenetic) and Passmore score (risk score), and c) correlating with progression and evolution to leukemia. Methods: Medical records of 56 children with cytopenias who were registered at the Hemato-oncology Unit, Hospital Roberto del Rio, were retrospectively reviewed from July 2000 to December 2007. Results: 13 patients met MDS diagnostic criteria (7.3% of the total hematological malignancies population). According to CCC classification, de novo MDS, refractory cytopenias with dysplasia and abnormal karyotype were the most frequent features. All patients with a high Passmore score progressed to leukemia. Conclusions: SMD classification in pediatrics patients remains difficult given the many differences with an adult population. CCC appears to be the most applicable for our pediatric population. High Passmore score correlated with leukemia development.

Palabras clave : Myelodysplastic syndrome; Pediatrics; CCC classification; Passmore score.

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