Leucemia aguda linfoblástica infantil y síndrome de Down: análisis de los protocolos SHOP/LAL-99 y 05 | Revista Médica Internacional sobre el Síndrome de Down

Article information

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 (Sociedades Españolas de Hematología y Oncología Pediátricas) 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

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 and Oncology Societies) 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

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Bibliografía

[1.]

P. Kaminker, R. Armando.

Síndrome de Down. Segunda parte: estudios genéticos y función del pediatra.

Arch Argent Pediatr., 106 (2008), pp. 334-340

http://dx.doi.org/10.1590/S0325-00752008000400009 | Medline

[2.]

H. Hasle, I.H. Clemmensen, M. Mikkelsen.

Risk of leukaemia and solid tumors in individuals with Down's syndrome.

Lancet., 355 (2000), pp. 165-169

http://dx.doi.org/10.1016/S0140-6736(99)05264-2 | Medline

[3.]

M. Schrappe, A. Reiter.

Long-term results of four consecutive trials in childhood ALL performed by the ALL-BFM study group from 1981 to 1995. Berlin-Frankfurt-Munster.

Leukemia., 14 (2000), pp. 2205-2222

Medline

[4.]

I. Badell, J. Cubells.

Experiencia de grupos cooperativos españoles en el tratamiento de la leucosis aguda linfoblástica infantil, pp. 406-409

[5.]

J. Cubells.

Protocolo de estudio y tratamiento de la leucemia aguda linfoblástica en pediatría (LAL/SHOP-99).

Rev Esp Pediatr., 57 (2001), pp. 523-533

[6.]

B. Zeller, G. Gustaffson.

Acute leukaemia in children with Down syndrome: a population-based Nordic study.

Br J Haematol., 128 (2005), pp. 797-804

http://dx.doi.org/10.1111/j.1365-2141.2005.05398.x | Medline

[7.]

D. Webb, I. Roberts.

Hematology of Down syndrome.

Arch Dis Child Fetal Neonatal Ed., 92 (2007), pp. F503-F507

http://dx.doi.org/10.1136/adc.2006.104638 | Medline

[8.]

M. Arico, O. Ziino.

Acute Lymphoblastic Leukemia and Down Syndrome: presenting features and treatment outcome in the experience of the Italian Association of Pediatric Hematology and Oncology (AIEOP).

Cancer., 113 (2008), pp. 515-521

http://dx.doi.org/10.1002/cncr.23587 | Medline

[9.]

B. Lange.

The management of neoplastic disorders of hematopoiesis in children with Down's syndrome.

Br J Haematol., 110 (2000), pp. 512-524

Medline

[10.]

M.C. Zwaan, D. Reinhardt.

Acute leukemias in children with Down syndrome.

Pediatr Clin N Am., 55 (2008), pp. 53-70

[11.]

G.V. Massey, A. Zipursky.

Aprospective study of the natural history of transient leukemia in neonates with Down syndrome: Children's Oncology Group study POG-9481.

Blood., 107 (2006), pp. 4606-4613

http://dx.doi.org/10.1182/blood-2005-06-2448 | Medline

[12.]

E. Forestier, S. Izraeli.

Cytogenetic features of acute lymphoblastic and myeloid leukemias in pediatric patients with Down syndrome: an iBFM-SG study.

Blood., 111 (2008), pp. 1575-1583

http://dx.doi.org/10.1182/blood-2007-09-114231 | Medline

[13.]

S. Fernández-Plaza, J. Sevilla.

Leucemia aguda en pacientes con síndrome de Down.

An Pediatr (Barc)., 61 (2004), pp. 515-519

Medline

[14.]

U. Creutzig, D. Reinhardt.

AML patients with Down syndrome have a high cure rate with AML-BFM therapy with reduced dose intensity.

Leukemia., 19 (2005), pp. 1355-1360

http://dx.doi.org/10.1038/sj.leu.2403814 | Medline

[15.]

D. Bercovich, I. Ganmore.

Mutations of JAK2 in acute lymphoblastic leukaemias associated with Down's syndrome.

Lancet., 372 (2008), pp. 1484-1492

http://dx.doi.org/10.1016/S0140-6736(08)61341-0 | Medline

[16.]

C. Mulligan, R. Collins-Underwood.

Rearrangement of CRLF2 in B-progenitor — and Down syndrome — associated acute lymphoblastic leukemia.

Nat Genet., 41 (2009), pp. 1243-1248

http://dx.doi.org/10.1038/ng.469 | Medline

[17.]

J.A. Whitlock, H.N. Sather.

Clinical characteristics and outcome of children with Down syndrome and acute lymphoblastic leukemia: a Children's Cancer Group study.

Blood., 106 (2005), pp. 4043-4049

http://dx.doi.org/10.1182/blood-2003-10-3446 | Medline

[18.]

J.M. Chessells, G. Harrison.

Down's syndrome and acute lymphoblastic leukaemia: clinical features and response to treatment.

Arch Dis Child., 85 (2001), pp. 321-325

Medline

[19.]

M. Dördelmann, M. Schrappe.

Down's syndrome in childhood acute lymphoblastic leukemia: clinical characteristics and treatment outcome in four consecutive BFM trials.

Leukemia., 12 (1998), pp. 645-651

Medline

[20.]

G. Lonnerhölm, B.M. Frost.

Vincristine pharmacokinetics in children with Down syndrome.

Pediatr Blood Cancer., 52 (2009), pp. 123-144

http://dx.doi.org/10.1002/pbc.21691 | Medline

[21.]

C. Bohndsted, M. Taskinen.

Poor treatment compliance in children with acute lymphoblastic leukemia and Down syndrome.

J Pediatr Hematol Oncol., 31 (2009), pp. 81-82

http://dx.doi.org/10.1097/MPH.0b013e3181923cb4 | Medline

☆

Este trabajo recibió un accésit del XI Premio Bienal de Investigación Ramon Trias Fargas 2007–2009.

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