Pediatric langerhans cell histiocytosis: single center experience over a 17-year period

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dc.contributor.authorDilek İnce
dc.contributor.authorBengü Demirağ
dc.contributor.authorGülcihan Özek
dc.contributor.authorAyşe Erbay
dc.contributor.authorRagıp Ortaç
dc.contributor.authorYeşim Oymak
dc.contributor.authorSerra Kamer
dc.contributor.authorYöntem Yaman
dc.contributor.authorSelcen Kundak
dc.contributor.authorCanan Vergin
dc.date.accessioned2019-10-26T19:41:58Z
dc.date.available2019-10-26T19:41:58Z
dc.date.issued2016
dc.departmentEge Üniversitesien_US
dc.description.abstractİnce D, Demirağ B, Özek G, Erbay A, Ortaç R, Oymak Y, Kamer S, Yaman Y, Kundak S, Vergin C. Pediatric langerhans cell histiocytosis: single center experience over a 17-year period. Turk J Pediatr 2016; 58: 349-355.This study aimed to analyze children with the diagnosis of Langerhans cell histiocytosis (LCH) who were diagnosed and treated between 1998-2015. Medical records were evaluated retrospectively for clinical and laboratory features, treatment details, and outcome. There were 20 patients, the median age of diagnosis was 37 months, M/F ratio: 1.5. Nine had single system (SS), 11 had multisystem (MS) LCH. Spontaneous regression occurred in three infants with skin limited LCH. Eight patients had risk organ involvement in MS-LCH group. The curettage alone was performed in only one case. Patients received LCH-II/ LCH-III based chemotherapy schema. Radiotherapy was performed to vertebral disease and residual craniofacial bone disease in four cases.The regression and relapse rates were 100% and 33% for SS-LCH. The regression and relapse rates were 73%, and 18% for MS-LCH. Two infants with MS-LCH died despite chemotherapy.Pulmonary and liver involvements affected outcome adversely in MS-LCH. Multidisciplinary treatment approaches are needed.en_US
dc.description.abstractİnce D, Demirağ B, Özek G, Erbay A, Ortaç R, Oymak Y, Kamer S, Yaman Y, Kundak S, Vergin C. Pediatric langerhans cell histiocytosis: single center experience over a 17-year period. Turk J Pediatr 2016; 58: 349-355.This study aimed to analyze children with the diagnosis of Langerhans cell histiocytosis (LCH) who were diagnosed and treated between 1998-2015. Medical records were evaluated retrospectively for clinical and laboratory features, treatment details, and outcome. There were 20 patients, the median age of diagnosis was 37 months, M/F ratio: 1.5. Nine had single system (SS), 11 had multisystem (MS) LCH. Spontaneous regression occurred in three infants with skin limited LCH. Eight patients had risk organ involvement in MS-LCH group. The curettage alone was performed in only one case. Patients received LCH-II/ LCH-III based chemotherapy schema. Radiotherapy was performed to vertebral disease and residual craniofacial bone disease in four cases.The regression and relapse rates were 100% and 33% for SS-LCH. The regression and relapse rates were 73%, and 18% for MS-LCH. Two infants with MS-LCH died despite chemotherapy.Pulmonary and liver involvements affected outcome adversely in MS-LCH. Multidisciplinary treatment approaches are needed.en_US
dc.identifier.endpage355en_US
dc.identifier.issn0041-4301
dc.identifier.issue4en_US
dc.identifier.startpage349en_US
dc.identifier.urihttps://app.trdizin.gov.tr/makale/TWpRek1UQTFOUT09
dc.identifier.urihttps://hdl.handle.net/11454/12342
dc.identifier.volume58en_US
dc.indekslendigikaynakTR-Dizinen_US
dc.language.isoenen_US
dc.relation.ispartofTurkish Journal of Pediatricsen_US
dc.relation.publicationcategoryMakale - Ulusal Hakemli Dergi - Kurum Öğretim Elemanıen_US]
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subjectPediatrien_US
dc.titlePediatric langerhans cell histiocytosis: single center experience over a 17-year perioden_US
dc.typeArticleen_US

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