Langerhans Cell Histiocytosis (LCH) in Egyptian Children: Does Reactivation Affect the Outcome?
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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 42 mo (range: 1.18 to 71 mo).
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 5 y 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.
KeywordsLCH Risk organs Response Reactivation Survival
The authors acknowledges Dr. Jean Donadieu, Pediatric Hemato Oncologist, Service d'Hémato-Oncologie Pédiatrique, Hopital Trousseau, Paris, France, for his effort in guiding his team during the preparation of this manuscript. His vast experience in Histiocytosis was the light that they followed for reaching the end of this work.
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