Clinical Characteristics and Treatment Outcome of Childhood Langerhans Cell Histiocytosis / 대한소아혈액종양학회지
Korean Journal of Pediatric Hematology-Oncology
; : 45-54, 2004.
Article
em Ko
| WPRIM
| ID: wpr-114801
Biblioteca responsável:
WPRO
ABSTRACT
PURPOSE:
We analyzed a cohort of patients with Langerhans cell histiocytosis (LCH) to understand the clinical findings, optimal management, and outcome of the disease.METHODS:
We performed a retrospective clinical study of LCH from January 1993 to August 2002 at Chonnam National University Hospital. All 39 patients with histologically proven histiocytosis were categorized into Class I (n=22), Class II (n=15) and Class III (n=2) by WHO classification.RESULTS:
There were 18 males and 21 females. Mean age at diagnosis was 3.2 years. The common clinical manifestations of Class I were soft tissue swelling, skin rash or nodule, otorrhea; and those of Class II were hepatosplenomegaly, fever, and respiratory symptoms. The most commonly involved organ of Class I was the skeleton; and that of Class II was bone marrow. Abnormal hematologic findings were found in 23 patients, especially in all Class II patients. Infectious etiology was documented in 5 Class II patients (CMV in 3, EBV in 1, mycoplasma in 1). Chemotherapy was given to 19 out of 22 Class I patients. Six of them showed complete remission. Four died during chemotherapy. The overall survival of Class I patients was 78% and that of Class II 63%. Poor prognostic factors of Class I were age 1.5 mg/dL.CONCLUSION:
The Langerhans cell histiocytosis is a heterogeneous disorder of significant morbidity and mortality. Early recognition and aggressive medical treatment might improve the survival rate.Palavras-chave
Texto completo:
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Base de dados:
WPRIM
Assunto principal:
Esqueleto
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Bilirrubina
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Medula Óssea
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Histiocitose
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Histiocitose de Células de Langerhans
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Taxa de Sobrevida
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Estudos Retrospectivos
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Estudos de Coortes
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Mortalidade
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Resultado do Tratamento
Tipo de estudo:
Diagnostic_studies
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Etiology_studies
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Incidence_studies
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Observational_studies
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Prognostic_studies
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Risk_factors_studies
Limite:
Female
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Humans
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Male
Idioma:
Ko
Revista:
Korean Journal of Pediatric Hematology-Oncology
Ano de publicação:
2004
Tipo de documento:
Article