Langerhans cell histiocytosis: 37 cases in a single Brazilian institution
Rev. bras. hematol. hemoter
; Rev. bras. hematol. hemoter;33(5): 353-357, Oct. 2011. tab
Article
em En
| LILACS
| ID: lil-606711
Biblioteca responsável:
BR408.1
ABSTRACT
OBJECTIVES:
To improve the level of 'definitive' diagnosis of Langerhans cell histiocytosis by immunohistochemical investigation of the CD1a surface antigen and to compare outcomes in respect to age, gender, stage of the disease, treatment response and level of diagnostic accuracy.METHODS:
A retrospective study was carried out of 37 children and adolescents with possible Langerhans cell histiocytosis between 1988 and 2008. The diagnoses were revisited using immunohistochemical investigations for CD1a, S-100 and CD68 in an attempt to reach definitive diagnoses for all cases.RESULTS:
Before the study, only 13 of 37 patients (35.1 percent) had a 'definitive' diagnosis; by the end of the study, this number rose to 25 patients (67.6 percent). All reviewed cases were positive for the CD1a antigen. Overall survival was 88.5 percent. Multisystem disease (Stage 2; n=19) and absence of response at the 6th week of therapy (n=5) were associated to significantly lower overall survival (p-value = 0.04 and 0.0001, respectively). All deaths occurred in patients with multisystem disease and organ dysfunction at diagnosis. Other potential prognostic factors were not significant. Reactivation episodes occurred in 75 percent of the patients with multisystem disease. Diabetes insipidus was the most common sequel (21.6 percent).CONCLUSION:
The level of diagnostic accuracy was increased through immunohistochemistry. The overall survival rate was similar to international multicentric studies. Multisystem disease and absence of response at six weeks of treatment were the most important unfavorable prognostic factors. The frequency of reactivation for patients with multisystem disease was higher than described in the literature, probably because maintenance chemotherapy was used only in two cases.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
LILACS
Assunto principal:
Otite
/
Histiocitose de Células de Langerhans
/
Diabetes Insípido
Tipo de estudo:
Clinical_trials
/
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Limite:
Adolescent
/
Child
/
Female
/
Humans
/
Male
País/Região como assunto:
America do sul
/
Brasil
Idioma:
En
Revista:
Rev. bras. hematol. hemoter
Assunto da revista:
HEMATOLOGIA
Ano de publicação:
2011
Tipo de documento:
Article
País de afiliação:
Brasil
País de publicação:
Brasil