Acute lymphoblastic leukemia in children and Down syndrome: Analysis of SHOP/ALL-’99 and ‘05 Protocols | Revista Médica Internacional sobre el Síndrome de Down (English Edition)

Article information

Abstract

Introduction and objective

Down syndrome bears a known predisposition to childhood leukemia. In regards to acute lymphoblastic leukemia (ALL), most international groups show poorer results when compared to non-Down patients.

Patients and methods

With this study we analyze the results obtained with Down syndrome patients and ALL younger than 18 years who were treated with SHOP (Spanish Pediatric Hematology Society) protocols for the past decade.

Results

Current data obtained from 1000 patients out of 32 centers confirm several aspects: those are related to acute leukemia showing clinical and biological low risk treats, thereof they may be categorized in low risk groups hence receive scheduled chemotherapy of moderate intensity. However, the number of infectious and toxic complications is greater than those for non-Down patients, therefore both overall survival (OS) and event free survival (EFS) are markedly affected.

Conclusions

The future aim is to optimize the knowledge on biological aspects of these leukemia, in order to determine those features to be acted upon to improve their outcome.

Keywords:

Acute lymphoblastic leukemia

Childhood leukemia

Down syndrome

Resumen

Introducción y objetivo

El síndrome de Down (SD) tiene una predisposición conocida a distintos tipos de leucemia infantil. En el caso de la leucemia aguda linfoblástica (LAL), la mayoría de autores refieren peores resultados con respecto a los pacientes no Down.

Pacientes y método

En el presente trabajo analizamos los resultados obtenidos en los pacientes con SD y LAL <18 años tratados según los protocolos del grupo SHOP (Sociedad Española de Hematología Pediátrica) durante la última década.

Resultados

Los datos obtenidos a partir de casi 1.000 pacientes que proceden de 32 centros, confirman diversos aspectos: se trata de leucemias agudas con características clínicas y biológicas de bajo riesgo, por lo que suelen estratificarse en grupos de riesgo bajo y reciben quimioterapias de intensidad moderada. Sin embargo, el número de complicaciones infecciosas y tóxicas es superior al de las de pacientes sin SD, por lo que tanto la supervivencia global (SG) como la supervivencia libre de eventos (SLE) se ven marcadamente afectadas.

Conclusiones

Debemos optimizar el conocimiento de la biología de estas leucemias para interpretar cuáles son los factores sobre los que podemos incidir para mejorar su pronóstico.

Palabras clave:

Leucemia aguda linfoblástica

Síndrome de Down

Leucemia infantil

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☆

T his study received a second prize of the 2007–2009 XI Premio Bienal de Investigación Ramon Trias Fargas (Ramon Trias Fargas Biennial Research Award).

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