title: Langerhans Cell Histiocytosis (LCH) in Egyptian Children: Does Reactivation Affect the Outcome?
abstract: OBJECTIVE
  To report a single centre outcome of management of Langerhans cell histiocytosis (LCH), a clonal disease with involvement of various body systems.
  
  
  METHODS
  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).
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Pediatrics, National Research Centre, Cairo, Egypt. mohamed.sedky@57357.com.|Department of Pediatric Oncology, Children Cancer Hospital, 1 Seket Al-Imam st., Al Sayyeda Zeinab, 26386, Cairo, Egypt. mohamed.sedky@57357.com.
authors.forename: Mohamed Sedky Mahmoud
authors.initials: MS
authors.lastname: Sedky
authors.affiliation: Department of Pediatric Oncology, National Cancer Institute, Cairo University, Cairo, Egypt.|Children Cancer Hospital, Cairo, Egypt.
authors.forename: Hany Abdel
authors.initials: HA
authors.lastname: Rahman
authors.affiliation: Children Cancer Hospital, Cairo, Egypt.|Department of Clinical Oncology, Menoufeya University, Cairo, Egypt.
authors.forename: Emad
authors.initials: E
authors.lastname: Moussa
authors.affiliation: Department of Pathology, Children Cancer Hospital, Cairo, Egypt.|National Cancer Institute, Cairo University, Cairo, Egypt.
authors.forename: Hala
authors.initials: H
authors.lastname: Taha
authors.affiliation: Department of Radiology, National Cancer Institute, Cairo University, Children Cancer Hospital, Cairo, Egypt.
authors.forename: Tarek
authors.initials: T
authors.lastname: Raafat
authors.affiliation: Department of Clinical Research, Children Cancer Hospital, Cairo, Egypt.
authors.forename: Omayma
authors.initials: O
authors.lastname: Hassanein
mesh_terms: D002648:Child; D002675:Child, Preschool; D004534:Egypt; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use; D006646:Histiocytosis, Langerhans-Cell / Q000188:drug therapy* / Q000401:mortality; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D011241:Prednisone / Q000627:therapeutic use; D011379:Prognosis; D012008:Recurrence; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome; D050257:Tubulin Modulators / Q000627:therapeutic use; D014747:Vinblastine / Q000627:therapeutic use
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D050257:Tubulin Modulators; D014747:Vinblastine; D011241:Prednisone; D008727:Methotrexate
keywords: LCH; Reactivation; Response; Risk organs; Survival