2007 Volume 21 Issue 5-6 Pages 289-295
There is considerable variation in the incidence of the late effects after Langerhans cell histiocytosis (LCH), however, about half of the children with LCH (22-64%) have some late sequelae. The most common late effects are endocrinological problems including central diabetes insipidus (15-50%) and growth hormone deficiency (5-20%). Other common effects were skeletal defects in 15% (2.5-42%) of LCH, dental problems in 3-30%, hearing loss in 3-16%, and other central nervous system dysfunction including cognitive dysfunction and cerebellar involvement in 2-14%. Health-related quality of life was adversely affected in > 50% of patients with multi-system LCH. The association of LCH and malignancy has been described in many reports before today. According to the Malignancy Registry of the Histiocytosis Society, 157 LCH-malignancy cases have been registered. Organ damage from LCH causes long term morbidity extending into adolescent and adult life. Carefully planned, multidisciplinary followup is essential to ensure early recognition of problems with appropriate interventions to reduce the impact on patients' quality of life.
