Langerhans cell histiocytosis. Ten-year experience in The National Institute of Cancer of Colombia
Keywords:
Langerhans cell histiocytosis, skin manifestationsAbstract
Introducción: Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of histiocytes characterized by a proliferation of abnormal and clonal Langerhans cells. The disease presents varied clinical manifestations. Descriptions of Hispanic patients have been scarce.
Methods: Clinical profile, disease-distribution and outcome of 34 cases of LCH from January 1988 to December 1999 is presented in this retrospective analysis based on the hospital records, with special interest in skin disorders.
Results: The age ranged from 4 months to 47 years, median age 3 years. Multisystem disease was documented in 16 (47%) patients. The most common type of skin lesion was papular rash in 8 (23.5%) patients. During a mean time follow-up period of 3.48 years, 7 (20.5%) patients died. Cases with localized or cutaneous limited disease received topical steroids, radiotherapy, cryosurgery, and oral steroids while most of cases with disseminated/multi-system disease were treated with prednisolone and, vinblastine or etoposide.
Conclusion: General outcome of the LCH in the National Cancer Institute of Colombia is similar to that described in other large series of patients.
Author Biographies
Santiago Andrés Ariza
Especialista en Dermatología. Fellow en Dermatología Oncológica. Instituto Nacional de Cancerología, Bogotá.
Andrés Felipe Cardona
Especialista en Epidemiología, Universidad del Rosario. Especialista en Medicina Interna. Especialista en Oncología Clínica. Instituto Nacional de Cancerología. Bogotá.
Xavier Rueda
Especialista en Dermatología Oncológica. Servicio de Dermatología Oncológica. Instituto Nacional de Cancerología. Bogotá.
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