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Lookup NU author(s): Dr Jane Salotti, Dr Kevin Windebank
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Purpose: Outcome review of paediatric Langerhans Cell Histiocytosis (LCH) in United Kingdom. Methods: The National Registry of Childhood Tumours at the Childhood Cancer Research Group records children with neoplasms and histiocytoses treated at paediatric oncology principal treatment centers (PTC) affiliated to the Children’s Cancer and Leukaemia Group. Retrospective review of registry records of all children (<15 years) diagnosed with LCH, between 1978–2007 was performed. Age, sex, systems affected at diagnosis and outcome were recorded. Results: 821 children were registered, 503 males, 318 females (ratio 1.6:1). The highest sex ratio was in the 10–14 years age group (2.2 males: 1 female). The annual number of cases increased over each 5 year period and was 22.7 in 1978–92 and 34.9 in 1993–2007, an increase of 54%. The majority were single system (69%), most commonly bone, while 28% were multi-system and unrecorded in 3%. Percentage of children registered who died within 5 years from diagnosis decreased with time, from 26% in 1978 –1982 to <1% in 2003–2007 (p<0.001). In addition to the 71 deaths within 5 years from diagnosis, there have been 9 deaths among those who have survived more than 5-years. Conclusions: This study represents the largest number of patients with LCH in a follow-up study to date. Increasing registration rate probably reflects increasing awareness of LCH, tendency for paediatric oncologists to care for children with LCH and an increasing propensity for children with good prognosis single-system LCH to be referred to PTCs and therefore included on the cancer Registry. Survival trends follow introduction of treatment protocol trials (LCH I-III) confirming importance of systematic treatment and supportive care. Collection of further data is planned using a questionnaire, based on that designed by the Late Effects Group of the Histiocyte Society, and will greatly add to our knowledge of disease reactivations and late effects.
Author(s): Holme H, Salotti J, Stiller C, Windebank K, Nanduri V
Publication type: Conference Proceedings (inc. Abstract)
Publication status: Published
Conference Name: Histiocyte Society 28th Annual Meeting
Year of Conference: 2013
Pages: E77-E77
ISSN: 1545-5009
Publisher: John Wiley & Sons, Inc.
URL: http://dx.doi.org/10.1002/pbc.24544
DOI: 10.1002/pbc.24544
Library holdings: Search Newcastle University Library for this item
Series Title: Pediatric Blood & Cancer
ISBN: 15455017