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Lookup NU author(s): Dr Jane Salotti
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Purpose: Outcome review of paediatric Haemophagocytosis Lymphohistiocytosis (HLH) in the 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 HLH, between 1978–2007 was performed. Age, sex, ethnicity and outcome were recorded. Results: 149 children were registered, 79 boys and 70 girls. The majority of cases (66%) were diagnosed in infancy. Ethnicity was recorded for 94% (51% Caucasian, and 34% South Asian). The proportions among infants under 1 year of age were 43% Caucasian and 41% South Asian. The number of cases increased markedly, with over 10 times the number in 2003 –2007 as 1978 –1982. Conversely, the percentage of children who died decreased with time. The proportions who died within 5 years from diagnosis were 92% in 1978–92, 70% in 1993 –2002 and 46% in 2003 –07 (p<0.001). Conclusions: This study represents the largest follow-up study of HLH to date. The high proportion of children registered in infancy possibly reflects greater incidence of Primary HLH. We believe increasing registration rate over time reflects an increasing awareness of HLH with diagnosis being made earlier. Improved survival followed introduction of treatment protocols HLH-1994 and HLH-2004. However, despite increased awareness, earlier diagnosis, improved therapy and supportive care, survival rates are still relatively poor with nearly 50% mortality among children diagnosed in the most recent period (2003–2007). This registry does not record details of genetic diagnosis, treatment and late sequelae in patients and this is the next planned step. A national registry of cases of HLH which includes diagnostic information, the treatment received and long term follow up data would enable further investigation of outcome and late-effects.
Author(s): Holme H, Salotti J, Stiller S, Dowse R, 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
Print publication date: 01/09/2013
Online publication date: 13/07/2013
ISSN: 1545-5017
Publisher: John Wiley & Sons, Inc.
URL: http://dx.doi.org/10.1002/pbc.24544
DOI: 10.1002/pbc.24544
Series Title: Pediatric Blood & Cancer
