Abstract
To report a single centre outcome of management of Langerhans cell histiocytosis (LCH), a clonal disease with involvement of various body systems. Retrospective analysis of 80 pediatric LCH patients at Children Cancer Hospital-Egypt between July 2007 and December 2011 was performed. Patients were stratified and treated according to LCH III protocol. The median follow up period was 42mo (range: 1.18 to 71mo). At wk 6 and 12, 'better' response was obtained in 61 (76%) and 74 (93%) patients respectively. Afterwards, reactivation occurred in 25 patients (38%), of them multiple episodes occurred in 5 patients (6.25%), managed by repetition of 1st line treatment for once or more. The 5y overall survival (OS) and event free survival (EFS) was 96.3 and 55% respectively. At last follow up, better status was reached in 70 patients, 3 in each 'intermediate' and 'worse' status. Three high risk patients died and one patient was lost to follow up. In a single Egyptian pediatric LCH experience, the response to treatment is satisfactory and survival remains the rule except in high risk organs disease that still needs a new molecule for salvage. However in multiple reactivations, patients do well with repetition of the 1st line of treatment with or without methotrexate.
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