Yıl: 2016 Cilt: 26 Sayı: 2 Sayfa Aralığı: 83 - 88 Metin Dili: Türkçe İndeks Tarihi: 29-07-2022

Langerhans Cell Histiocytosis in Children: A Single Center Experience from Turkey

Öz:
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 (%88.8). Bunu hepatosplenomegali (%44.4), lenfadenopati (%44.4), sarılık (%33.3), seboreik dermatit (%33.3) takip etmekte idi. Dokuz olgudan üçünde (%33.3) tek sistem tutulumu (1 tanesi tek odaklı, 2 tanesi çok odaklı), 6'sında (%66,6) çoklu sistem tutulumu [2 tanesi (%22.2) risk organ tutulumsuz, 4 tanesi (%44.4) risk organ tutulumlu] mevcuttu. 6 haftalık tedavinin sonunda; 5 hastada (%55.5) iyi cevap, 1 hastada (%11.1) orta cevap ve 3 (%33.3) hastada kötü cevap alındı. Gidişat açısından değerlendirildiğinde; 5 hasta (55.5%) tam remisyona girmiş, 3 (%33.3) 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.
Anahtar Kelime:

Konular: Onkoloji

Çocuklarda Langerhans Hücreli Histiyositoz: Türkiye'den Tek Merkez Deneyimi

Öz:
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 (%88,8), followed by hepatosplenomegaly (%44.4), lymphadenopathy (%44.4), seborrheic dermatitis (33,3%) and jaundice (33.3%). Of the 9 patients, 3 (33.3%) had single system disease [1 (11.1%) had unifocal while 2 (22.2%) had multifocal disease], 6 (66.6%) had multisystem disease [2 (22.2%) had risk organ negative and 4 (44.4%) had risk organ positive]. At the end of the 6th week of therapy, 5 patients (55.5%) had all the dead children had better response, 1 (11.1%) had an intermediate response while 3 (33.3%) had worse response. In terms of outcome, 5 (55%) patients were in complete remission, 3 (33%) 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.
Anahtar Kelime:

Konular: Onkoloji
Belge Türü: Makale Makale Türü: Araştırma Makalesi Erişim Türü: Erişime Açık
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APA Tokgöz H, ÇALISKAN Ü (2016). Langerhans Cell Histiocytosis in Children: A Single Center Experience from Turkey. , 83 - 88.
Chicago Tokgöz Hüseyin,ÇALISKAN ÜMRAN Langerhans Cell Histiocytosis in Children: A Single Center Experience from Turkey. (2016): 83 - 88.
MLA Tokgöz Hüseyin,ÇALISKAN ÜMRAN Langerhans Cell Histiocytosis in Children: A Single Center Experience from Turkey. , 2016, ss.83 - 88.
AMA Tokgöz H,ÇALISKAN Ü Langerhans Cell Histiocytosis in Children: A Single Center Experience from Turkey. . 2016; 83 - 88.
Vancouver Tokgöz H,ÇALISKAN Ü Langerhans Cell Histiocytosis in Children: A Single Center Experience from Turkey. . 2016; 83 - 88.
IEEE Tokgöz H,ÇALISKAN Ü "Langerhans Cell Histiocytosis in Children: A Single Center Experience from Turkey." , ss.83 - 88, 2016.
ISNAD Tokgöz, Hüseyin - ÇALISKAN, ÜMRAN. "Langerhans Cell Histiocytosis in Children: A Single Center Experience from Turkey". (2016), 83-88.
APA Tokgöz H, ÇALISKAN Ü (2016). Langerhans Cell Histiocytosis in Children: A Single Center Experience from Turkey. Uluslararası Hematoloji-Onkoloji Dergisi, 26(2), 83 - 88.
Chicago Tokgöz Hüseyin,ÇALISKAN ÜMRAN Langerhans Cell Histiocytosis in Children: A Single Center Experience from Turkey. Uluslararası Hematoloji-Onkoloji Dergisi 26, no.2 (2016): 83 - 88.
MLA Tokgöz Hüseyin,ÇALISKAN ÜMRAN Langerhans Cell Histiocytosis in Children: A Single Center Experience from Turkey. Uluslararası Hematoloji-Onkoloji Dergisi, vol.26, no.2, 2016, ss.83 - 88.
AMA Tokgöz H,ÇALISKAN Ü Langerhans Cell Histiocytosis in Children: A Single Center Experience from Turkey. Uluslararası Hematoloji-Onkoloji Dergisi. 2016; 26(2): 83 - 88.
Vancouver Tokgöz H,ÇALISKAN Ü Langerhans Cell Histiocytosis in Children: A Single Center Experience from Turkey. Uluslararası Hematoloji-Onkoloji Dergisi. 2016; 26(2): 83 - 88.
IEEE Tokgöz H,ÇALISKAN Ü "Langerhans Cell Histiocytosis in Children: A Single Center Experience from Turkey." Uluslararası Hematoloji-Onkoloji Dergisi, 26, ss.83 - 88, 2016.
ISNAD Tokgöz, Hüseyin - ÇALISKAN, ÜMRAN. "Langerhans Cell Histiocytosis in Children: A Single Center Experience from Turkey". Uluslararası Hematoloji-Onkoloji Dergisi 26/2 (2016), 83-88.