Clinical Features and Disease Course of Langerhans' Cell Histiocytosis

Park, Sang Jin; Im, Ho Joon; Lee, Hahng

Abstract

BACKGROUND: The purpose of our investigation was to evalute response to therapy and prognosis according to clinical and pathologic stage.
METHODS
Fourteen children who had LCH were diagnosed between Jan., 1987 and Dec., 1996. Of the 14 patients, 3 patients with only skin involvement were excluded from this study. The medical records of 11 patients were reviewed for organ involvement at diagnosis, treatment, disease course, and late sequelae.
Results
1) As for the clinical stages, 4 patients were in stage I, 4 in stage II, one in stage III, and 2 in stage IV. As for the pathologic stages, four had monostotic disease, 2 polyostotic disease and 5 multisystemic disease. 2) All six patients with monostotic and polyostotic disease had a complete response to the therapy and remain alive free of disease. Three of the five with multisystemic disease had a complete response to the therapy, while two had a progression of disease. 3) Sequelae were observed in 3 patients including 2 with diabetes insipidus and 1 with hepatic and pulmonary dysfunctions. 4) The median follow-up time from diagnosis for all patients was 3.5 years. The Kaplan-Meier estimate of survival rate is 91%, with estimated EFS of 81.8% at 5 years.
CONCLUSION
The clinical outcome of LCH patients with single system involvement including monostotic and polyostotic diseases were favorable and all remains alive free of disease. Multisystemic disease was associated with several unfavorable prognostic features including diagnosis at age <2 year, dysfunction of involved organs and advanced clinical stage.