Nanduri Vasanta Rao, Pritchard Jon, Levitt Gill, Glaser Adam W
Department of Haematology/Oncology, Great Ormond Street Hospital for Children, London, and Yorkshire Regional Centre for Paediatric Oncology and Haematology, St James University Hospital, Leeds, UK.
Eur J Cancer. 2006 Oct;42(15):2563-9. doi: 10.1016/j.ejca.2006.05.031. Epub 2006 Sep 7.
Langerhans' cell histiocytosis, a clonal multisystem disorder, can affect children or adults resulting in long term sequelae. However, the overall morbidity for survivors has not been formally determined.
We performed a cross-sectional study of 40 unselected long term survivors of childhood multisystem Langerhans cell histiocytosis, involving clinical examination, health-related quality of life assessment, brain imaging, neuropsychometry, endocrine assessment, respiratory function tests and audiometry. A specific 'morbidity score' was devised to measure outcome.
Seventy-five percent of patients had detectable long term sequelae, hypothalamic-pituitary dysfunction (50%), cognitive dysfunction (20%) and cerebellar involvement (17.5%) being the most common. Half had moderate to severe morbidity, and the worst-affected patients were unable to lead an independent adult life. Health-related quality of life, which correlated well with the morbidity score (p<0.001), was adversely affected in >50% of patients.
Organ damage from multisystem Langerhans cell histiocytosis causes long term morbidity extending into adult life. Carefully planned, multidisciplinary follow up is essential to ensure early recognition of problems with appropriate interventions to reduce the impact on patients' 'quality of life'.
