Langerhans Cell Histiocytosis in Children: A Single Center Experience from Turkey
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Dosyalar
Tarih
2016
Yazarlar
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Bu çalışmanın amacı, merkezimizde Langerhans hücreli histiyositoz (LCH) tanısı alan çocuklar ile ilgili deneyimlerimizi sunmaktadır. LCH tanılı çocukların tıbbi kayıtları, hastaların cinsiyeti, tanı yaşı, klinik bulgular, risk grupları, tedavi rejimleri ve gidişat açısından değerlendirilmek üzere incelendi. Medyan yaşı 22 ay olan 9 hasta çalışmaya dâhil edildi. En sık görülen klinik bulgu kemik lezyonlarıydı. Bunu hepatosplenomegali, lenfadenopati, sarılık, seboreik dermatit takip etmekte idi. Dokuz olgudan üçünde tek sistem tutulumu (1 tanesi tek odaklı, 2 tanesi çok odaklı), 6'sında çoklu sistem tutulumu [2 tanesi risk organ tutulumsuz, 4 tanesi risk organ tutulumlu] mevcuttu. 6 haftalık tedavinin sonunda; 5 hastada iyi cevap, 1 hastada orta cevap ve 3 hastada kötü cevap alındı. Gidişat açısından değerlendirildiğinde; 5 hasta tam remisyona girmiş, 3 hasta ölmüş, bir hasta takipten çıkmıştı. Ölen çocukların hepsi çoklu sistem tutulumlu risk grubunda olan ve 6 haftalık tedaviye kötü tedavi cevabı olan hastalardı. LCH'li çocuklarda prognozu belirleyen en önemli faktörler; risk organ tutulumu, çoklu sistem tutulumu ve 6.hafta tedavisine olan cevaptır. Risk organ tutulumu olan hastalarda prognoz kötü olduğu için yeni ilaçlar veya kök hücre nakli gibi yeni tedavi yaklaşımlarına ihtiyaç vardır.
The aim of the study is to present the experience of children diagnosed with Langerhans cell histiocytosis (LCH) at our center. Medical records of children with LCH were screened in terms of gender, age at diagnosis, clinical findings, risk groups, treatment modalities and outcome. The study included 9 patients with a median age of 22 months. The most common clinical finding was bone lesions, followed by hepatosplenomegaly, lymphadenopathy, seborrheic dermatitis and jaundice. Of the 9 patients, 3 had single system disease had unifocal while 2 had multifocal disease], 6 had multisystem disease had risk organ negative and 4 had risk organ positive. At the end of the 6th week of therapy, 5 patients had all the dead children had better response, 1 had an intermediate response while 3 had worse response. In terms of outcome, 5 patients were in complete remission, 3 patients were dead, and 1 patient was lost to follow up. All dead children had multisystem disease with risk organ involvement and were worse responders at the 6th week of therapy. In children with LCH, the most important factors determining prognosis were risk organ involvement, multisystem disease and response at 6th week of treatment. New treatment modalities such as new drugs or stem cell transplantation are required for children with risk organ positive multisystem disease, as the prognosis remains poor.
The aim of the study is to present the experience of children diagnosed with Langerhans cell histiocytosis (LCH) at our center. Medical records of children with LCH were screened in terms of gender, age at diagnosis, clinical findings, risk groups, treatment modalities and outcome. The study included 9 patients with a median age of 22 months. The most common clinical finding was bone lesions, followed by hepatosplenomegaly, lymphadenopathy, seborrheic dermatitis and jaundice. Of the 9 patients, 3 had single system disease had unifocal while 2 had multifocal disease], 6 had multisystem disease had risk organ negative and 4 had risk organ positive. At the end of the 6th week of therapy, 5 patients had all the dead children had better response, 1 had an intermediate response while 3 had worse response. In terms of outcome, 5 patients were in complete remission, 3 patients were dead, and 1 patient was lost to follow up. All dead children had multisystem disease with risk organ involvement and were worse responders at the 6th week of therapy. In children with LCH, the most important factors determining prognosis were risk organ involvement, multisystem disease and response at 6th week of treatment. New treatment modalities such as new drugs or stem cell transplantation are required for children with risk organ positive multisystem disease, as the prognosis remains poor.
Açıklama
WOS:000388274800003
Anahtar Kelimeler
Langerhans cell histiocytosis, Child, Langerhans hücreli histiyositoz, Çocuk
Kaynak
Uluslararası Hematoloji-Onkoloji Dergisi
WoS Q Değeri
Q4
Scopus Q Değeri
Q4
Cilt
26
Sayı
2
Künye
Tokgöz, H., Çalışkan, Ü. (2016). Langerhans cell histiocytosis in children: A Single center experience from Turkey. Uhod - Uluslararasi Hematoloji-Onkoloji Dergisi, 26, 2, 83-88.
