[Results of immunosuppressive therapy in children with severe aplastic anaemia. Report by the Polish Paediatric Leukaemia and Lymphoma Study Group]. / Efekty leczenia immunosupresyjnego ciezkiej postaci anemii aplastycznej u dzieci. Raport polskiej pediatrycznej grupy ds. leczenia bialaczek i chloniaków.
Med Wieku Rozwoj
; 10(3 Pt 1): 832-9, 2006.
Article
em Pl
| MEDLINE
| ID: mdl-17317914
ABSTRACT
INTRODUCTION:
Bone marrow transplantation from HLA identical family donors is the treatment of choice for children with severe aplastic anaemia (SAA). When no donor is available, combined immunosuppressive therapy is given.AIM:
Evaluation of results of immunosuppressive therapy in children with severe aplastic anaemia. MATERIAL ANDMETHODS:
SAA was diagnosed in 85 children (31 girls, 54 boys) aged 2-17.5 years in the eleven centres of the Polish Paediatric Leukaemia and Lymphoma Study Group (PPLLSG) in Poland between 1993-2003 years. All patients received protocol of the Severe Aplastic Anaemia Working Party of the Europe Bone Marrow Transplant (EBMT) antilymphocyte globulin or antithymocyte globulin, cyclosporin A, prednisolone and granulocyto- or granulocyto-macrophagic-cell stimulation factor was additionally administered during deep neutropenia. Haematological response was evaluated on day 84, 112 or 180 of the therapy.RESULTS:
complete remission occurred in 43 patients (50.5%), partial remission in 22 (25.4%), no response was obtained in 20 children (23.7%) in 180 day of the therapy. Period of observation was from 12 months to 10.5 years. During this time relapse occurred in 6 patients (7%). We observed 16 deaths 7 early during the first 3 months of immunosuppressive therapy (IS) and 9 after the first 3 months of IS.CONCLUSION:
the actual survival at 10-years, after immunosuppressive therapy is 81.2% in our group. Transformation to leukaemia or myelodysplastic syndrome (MDS) was not observed in any of our patients. We observed one case with paroxysmal nocturnal haemoglobinuria (PNH).
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Bases de dados:
MEDLINE
Assunto principal:
Terapia de Imunossupressão
/
Imunossupressores
/
Anemia Aplástica
Tipo de estudo:
Guideline
/
Observational_studies
/
Risk_factors_studies
Limite:
Adolescent
/
Child
/
Child, preschool
/
Female
/
Humans
/
Male
País/Região como assunto:
Europa
Idioma:
Pl
Revista:
Med Wieku Rozwoj
Assunto da revista:
PEDIATRIA
Ano de publicação:
2006
Tipo de documento:
Article
País de afiliação:
Polônia