Treatment of pediatric myelodysplastic syndromes and juvenile myelomonocytic leukemia: the Brazilian experience in the past decade.
BACKGROUND
Therapy strategies for myelodysplastic syndromes (MDS) and juvenile myelomonocytic leukemia (JMML) vary considerably.


OBJECTIVE
To review the treatment of Brazilian children who were diagnosed with MDS or JMML in the past decade and reported to the Brazilian Cooperative Group on Pediatric Myelodysplastic Syndromes (BCG-MDS-PED).


RESULTS
Of 173 children reported to the BCG-MDS-PED from January 1997 to January 2003 with a suspected diagnosis of MDS or JMML, 91 had the diagnosis confirmed after central review of the bone marrow aspirate and biopsy. Information on previous treatments was available for 78 MDS/JMML patients. Treatment varied from different schedules of low-dose (14%) and standard-dose chemotherapy (50%), granulocyte-colony-stimulating factor (G-CSF 7%), interferon (5%), steroids (2%) and erythropoietin (2%) to allogeneic stem-cell transplantation (SCT) (14%). No survival advantage could be demonstrated based on Hasle's classification or based on treatment.


CONCLUSIONS
This report reflects the current practice in treating Brazilian children with MDS/JMML without specific Cooperative Group guidelines. Treatment modalities were very heterogeneous. The strategies for implementing a national protocol should consider international guidelines and focus on local experience and available resources.
Valera
Elvis Terci
ET
Departamento de Puericultura e Pediatria, Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Av. Bandeirantes, 3900 Monte Alegre, CEP 14049-900, Brazil. elvisvalera@hotmail.com
Latorre
Maria do Rosário Dias
Mdo R
Mendes
Wellington Luiz
WL
Seber
Adriana
A
Lee
Maria Lucia Martino
ML
de Paula
Mario José Aguiar
MJ
Loggetto
Sandra Regina
SR
Velloso
Elvira
E
Niero-Melo
Ligia
L
Lopes
Luiz Fernando
LF
D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001938:Brazil; D002648:Child; D002675:Child, Preschool; D020732:Cytogenetic Analysis; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007223:Infant; D015477:Leukemia, Myelomonocytic, Chronic / Q000401:mortality / Q000628:therapy*; D008297:Male; D009190:Myelodysplastic Syndromes / Q000401:mortality / Q000628:therapy*; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome