Nanduri, Vasanta Rao; (2002) Long term sequelae of multisystem Langerhans' cell histiocytosis. Doctoral thesis (Ph.D.), University College London (United Kingdom).

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Abstract

Langerhans' cell histiocytosis (LCH), a chronic granulomatous disorder, can involve one or more organs/tissues including bone, skin, lungs, liver, spleen, bone marrow, pituitary gland and brain. Long term sequelae involving these organs have been reported, but their true prevalence is unknown. In order to assess long term outcome in survivors of multisystem LCH, we performed a cross-sectional study of 40 patients, all of whom were more than 5 years from treatment. Most of the patients had had involvement of bone and/or skin, with other organs being affected less often. They had received a wide range of treatments, including surgery, steroids, radiotherapy and chemotherapy. The study involved clinical examination, MRI scan of brain, endocrine function tests, neuropsychometry, respiratory function tests and audiometry. Most patients had one or more long term sequelae. Half of the patients had endocrine abnormalities, ranging from isolated diabetes insipidus to panhypopituitarism. Brain involvement, including cerebellar involvement, was the most worrying problem, occurring in 10 patients, with severe abnormalities in seven. New findings include the presence of significant learning deficit in 20% of patients, psychological and behavioural abnormalities in 11 patients (27.5%), and an acquired abnormality of the skull base, basilar invagination, in 8 patients (20%). A specific morbidity score was devised and provided an objective measure of outcome. Using this scale only 10 patients (25%) had no sequelae. Eleven (27.5%) had mild impairment which required no specific treatment, 9 (22.5%) had moderate disability, including diabetes insipidus, growth hormone insufficiency and moderate hearing loss, while 10 (25%) had severe disabilities such as panhypopituitarism, learning difficulty, motor deficit and psychological abnormalities resulting in significant handicap and inability to lead an independent adult life. We assessed the Health-Related Quality of Life and found that this correlated with the Morbidity score. Both these measures can easily be applied to any patient with LCH and can be incorporated into long term follow up studies. In conclusion, long term sequelae are more common in survivors of multisystem LCH than previously recognised and cause significant long term morbidity. An important implication of the work presented in this thesis is that carefully planned regular long-term follow up is essential for all patients 'cured' of LCH to ensure that sequelae are recognised early and the appropriate interventions made to improve the patients' "quality of life".

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