Langerhans cell histiocytosis: a 16-year experience.
OBJECTIVE
To describe the clinical course of Langerhans cell histiocytosis and to compare its outcome according to age, staging of the disease and treatment response.


METHODS
Retrospective analysis of data on 33 children with Langerhans cell histiocytosis followed at Hospital das Clínicas, Universidade Federal de Minas Gerais, Brazil, between 1988 and 2004.


RESULTS
Age at diagnosis ranged from 2 months to 16 years (median: 2.5 years). Seventeen children were male. The follow-up period varied from 21 days to 16.2 years (median: 3.4 years). The most common clinical manifestations at diagnosis were osteolytic lesions, enlarged lymph nodes and skin lesions. The overall survival rate for the whole group was 86.1% at 16 years (95%CI 66.6-94.6%). Deaths occurred in patients with multisystem disease and organ dysfunction at diagnosis. Those patients who had a "better" response to treatment in the sixth week were likely to have a significantly higher overall survival rate than those who showed disease progression. Overall survival rate was significantly higher for patients with single-system disease. The disease-free survival rate for the whole group was 30.9% at 16 years (95%CI 15.6-47.5%), and was significantly higher for those with single-system disease. Age groups were not associated with different disease-free survival rates. Diabetes insipidus was the most common sequela. No cases of secondary neoplasms were observed.


CONCLUSIONS
The clinical manifestations of Langerhans cell histiocytosis vary widely, with a high relapse rate and low mortality rate.
Campos
Márcia Kanadani
MK
Hospital das Clínicas, Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, MG, Brazil.
Viana
Marcos Borato
MB
de Oliveira
Benigna Maria
BM
Ribeiro
Daniel Dias
DD
Silva
Cláudia Márcia de Resende
CM
D000293:Adolescent; D000911:Antibodies, Monoclonal; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis* / Q000188:drug therapy / Q000401:mortality; D006801:Humans; D007155:Immunologic Factors; D007223:Infant; D008297:Male; D011379:Prognosis; D012008:Recurrence; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome
D000911:Antibodies, Monoclonal; D007155:Immunologic Factors