Outcome Comparison of Pediatric Langerhans Cell Histiocytosis Treated with Modified DAL-HX83/90 or JLSG-96 Protocol / 中国实验血液学杂志
Journal of Experimental Hematology
;
(6): 1190-1195, 2016.
Artigo
em Chinês
| WPRIM
| ID: wpr-246793
ABSTRACT
<p><b>OBJECTIVE</b>To analyze and compare the clinical features, treatment and prognosis of 31 children with Langerhans cell histiocytosis(LCH) treated with modified DAL-HX83/90 or JLSG-96 protocol.</p><p><b>METHODS</b>The clinical features, treatment and prognosis of 31 children with Langerhans cells admitted in our hospital from January 2005 to December 2014 were analyzed retrospectively. The outcome of patients treated with modified DAL-HX83/90 or JLSG-96 protocols were compared by using the Kaplan-Meier survival curve. Among 31 children with LCH, 19 males and 12 females, 12 younger than 2 years old, and 19 older than 2 years old. LCH usually affected skeleton system(77.4%), skin(42.0%), liver(29.0%), spleen(19.4%), hematopoietic system(12.9%). The most common misdiagnoses were upper respiratory tract infection, malignancies, focal infection, and eczema.</p><p><b>RESULTS</b>Response rate at week 6 was 76.9% in modified DAL-HX83/90 group and 94.1% in JLSG-96 group respectively, and no significant differences had been found between 2 groups. The 1-and 3-year overall survival rates of the patients treated with JLSG-96 protocol were 100% and 83.3%±15.2% respectively, while The 1-, 3-,5-year overall survival rates of those patients treated with the modified DAL-HX83/90 protocol were 70%±14.5%. The 1-and 3-year event-free rates of children treated with JLSG-96 protocol were 73.3%±11.4% and 66.7%±12.2%, respectively, while the 1-, 3-, 5-year event-free rates of those treated with modified DAL-HX83/90 protocol were 50%±15.8%, 40%±15.6% and 26.7%±15% respectively. No differences were found between the 2 groups for OS or EFS.</p><p><b>CONCLUSION</b>JLSG-96 protocol shows a better prognosis, and the risk of secondary malignancy caused by etoposide can be avoided. Early diagnosis of refractory LCH, specified grouping strategy and prolonged maintenance therapy may contribute to enhancing the EFS rates and reducing relapses.</p>
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Histiocitose de Células de Langerhans
/
Taxa de Sobrevida
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Estudos Retrospectivos
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Resultado do Tratamento
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Estimativa de Kaplan-Meier
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Recidiva Local de Neoplasia
Tipo de estudo:
Guia de Prática Clínica
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Estudo observacional
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Estudo de rastreamento
Limite:
Criança
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Criança, pré-escolar
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Feminino
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Humanos
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Lactente
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Masculino
Idioma:
Chinês
Revista:
Journal of Experimental Hematology
Ano de publicação:
2016
Tipo de documento:
Artigo
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