Yıl: 2007 Cilt: 24 Sayı: 2 Sayfa Aralığı: 75 - 79 Metin Dili: Türkçe İndeks Tarihi: 29-07-2022

Retrospective analysis of seven patients with adult-onset langerhans cell histiocytosis syndromes: A single center experience

Öz:
Langerhans cell histiocytosis sendromları geniş spektrumlu bir hastalık grubu olarak tanımlanır ve bu grupta Eosionophilic Granuloma, Letterer-Siewe syndrome ve Hand-Schuller-Christian Disease gibi hastalıklar bulunur. Bu hastalıklar genelde erken yaşta görülme eğilimindedir ve erişkin yaşta nadirdirler. Erişkin hastalar için standart bir tedavileri olmamakla birlikte 2-chlorodeoxyadenosine (Cladribine)’nin tedavide etkin olduğu bildirilmektedir. Bu çalışmada hastanemizde tanı alan ve takip edilen Langerhans cell histiocytosis hastaları geriye dönük olarak değerlendirilmiştir. Yedi hasta (4 erkek, 3 kadın) 1995–2005 yılları arasında merkezimizde takip edilmiştir. Ortalama yaş 27.5 (18–40) yıl olarak hesaplanmıştır. Başlıca şikâyetler; çoklu alanlarda kemik ağrısı (%100), polidipsi (%28), akciğer infiltrasyonu (%14), oral mukozal infiltrasyon (%14), ve kranial sinir tutulumu (%14) olarak kaydedilmiştir. İki hasta Hand-Schuller-Christian Disease, diğerleri Eosinophilic Granuloma olarak kabul edilmiştir. Hiçbir hastada kemik iliği ve akciğer dışı organ tutulumu saptanmamıştır. Kemik tutulumu, alan sayısı 1’den 7’ye kadar değişmekle beraber, en sık klinik bulgu olmuştur. Tüm hastalar halen yaşamaktadır. Bir hasta dışında tüm hastalar radyoterapi almış ve bir hasta vincristine ve dexamethasone ile tedavi edilmeye başlanmış ve tedavisi cladribine ile tamamlanmıştır. Yedi hastanın 2’si radyoterapi ve kemoterapi ile kombine tedavi edilmiştir. Kalan 4 hasta ise sadece radyoterapi ile tedavi edilmiştir. Tedavi sonrası tüm hastalar asemptomatik hale gelmiştir. Hiçbir hastada grade 3-4 hematolojik ve diğer sistem toksisiteleri gelişmemiştir İki hastada yeni kemik tutulumu tarzında reaktivasyon saptanmış ve bunlar radyoterapiye iyi yanıt vermiştir. Erişkin yaş grubunda Langerhans cell histiocytosis sendromları nispeten benin bir gidişat izlemekte ve radyoterapi ya da kemoterapi ile başarılı şekilde tedavi edilebilmektedir.
Anahtar Kelime: Erişkin Seyir Histiyositoz, langerhans-hücreli Geriyedönük çalışma

Konular: Hematoloji

Erişkin langerhans hücreli histiositoz sendromlu hastaların retrospektif incelenmesi

Öz:
granuloma, Letterer-Siwe syndrome and Hand-Schüller-Christian disease. These disorders have been mainly diagnosed at early ages of life and are relatively rare entities in adult age groups. In this study, we aimed to retrospectively evaluate the patients with Langerhans cell histiocytosis followed-up in our hospital. Seven patients were treated between 1995 and 2005. Median age of patients was 27.5 (18-40) years. Main complaints were classified as bone pain in multiple sites (100%), polydipsia (28%), lung infiltration (14%), oral mucosal infiltration (14%), and cranial nerve infiltration (14%). Two patients were diagnosed as Hand-Schüller-Christian disease, and the others were accepted as eosinophilic granuloma. There was no bone marrow or any other organ infiltration except lung infiltration in one patient. Bone infiltration was the prominent sign in all patients with a minimum of one to maximum of seven different sites. All patients were alive during the follow-up period. All patients were treated with radiotherapy except one patient treated with chemotherapy regimen started with vincristine plus dexamethasone and continued with cladribine. Three of seven patients were treated with combined modality, one patient with only chemotherapy and the others with only radiotherapy. There was no grade 3-4 hematological or systemic side effects of treatment. Relapses were detected in only two patients as new bone infiltrations which responded completely to radiotherapy. Langerhans cell histiocytosis syndromes have a relatively benign course in adult patients and can be treated with either radiotherapy or chemo-radiotherapy successfully.
Anahtar Kelime: Retrospective Studies Adult Prognosis Histiocytosis, Langerhans-Cell

Konular: Hematoloji
Belge Türü: Makale Makale Türü: Araştırma Makalesi Erişim Türü: Erişime Açık
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APA Sahin F, ÖZEN P, vural f, Kamer S, ARGIN M, Anacak Y, HEKİMGİL M, SAYDAM G (2007). Retrospective analysis of seven patients with adult-onset langerhans cell histiocytosis syndromes: A single center experience. , 75 - 79.
Chicago Sahin Fahri,ÖZEN Pınar Kezban,vural filiz,Kamer Serra,ARGIN MEHMET,Anacak Yavuz,HEKİMGİL Mine,SAYDAM Güray Retrospective analysis of seven patients with adult-onset langerhans cell histiocytosis syndromes: A single center experience. (2007): 75 - 79.
MLA Sahin Fahri,ÖZEN Pınar Kezban,vural filiz,Kamer Serra,ARGIN MEHMET,Anacak Yavuz,HEKİMGİL Mine,SAYDAM Güray Retrospective analysis of seven patients with adult-onset langerhans cell histiocytosis syndromes: A single center experience. , 2007, ss.75 - 79.
AMA Sahin F,ÖZEN P,vural f,Kamer S,ARGIN M,Anacak Y,HEKİMGİL M,SAYDAM G Retrospective analysis of seven patients with adult-onset langerhans cell histiocytosis syndromes: A single center experience. . 2007; 75 - 79.
Vancouver Sahin F,ÖZEN P,vural f,Kamer S,ARGIN M,Anacak Y,HEKİMGİL M,SAYDAM G Retrospective analysis of seven patients with adult-onset langerhans cell histiocytosis syndromes: A single center experience. . 2007; 75 - 79.
IEEE Sahin F,ÖZEN P,vural f,Kamer S,ARGIN M,Anacak Y,HEKİMGİL M,SAYDAM G "Retrospective analysis of seven patients with adult-onset langerhans cell histiocytosis syndromes: A single center experience." , ss.75 - 79, 2007.
ISNAD Sahin, Fahri vd. "Retrospective analysis of seven patients with adult-onset langerhans cell histiocytosis syndromes: A single center experience". (2007), 75-79.
APA Sahin F, ÖZEN P, vural f, Kamer S, ARGIN M, Anacak Y, HEKİMGİL M, SAYDAM G (2007). Retrospective analysis of seven patients with adult-onset langerhans cell histiocytosis syndromes: A single center experience. Turkish Journal of Hematology, 24(2), 75 - 79.
Chicago Sahin Fahri,ÖZEN Pınar Kezban,vural filiz,Kamer Serra,ARGIN MEHMET,Anacak Yavuz,HEKİMGİL Mine,SAYDAM Güray Retrospective analysis of seven patients with adult-onset langerhans cell histiocytosis syndromes: A single center experience. Turkish Journal of Hematology 24, no.2 (2007): 75 - 79.
MLA Sahin Fahri,ÖZEN Pınar Kezban,vural filiz,Kamer Serra,ARGIN MEHMET,Anacak Yavuz,HEKİMGİL Mine,SAYDAM Güray Retrospective analysis of seven patients with adult-onset langerhans cell histiocytosis syndromes: A single center experience. Turkish Journal of Hematology, vol.24, no.2, 2007, ss.75 - 79.
AMA Sahin F,ÖZEN P,vural f,Kamer S,ARGIN M,Anacak Y,HEKİMGİL M,SAYDAM G Retrospective analysis of seven patients with adult-onset langerhans cell histiocytosis syndromes: A single center experience. Turkish Journal of Hematology. 2007; 24(2): 75 - 79.
Vancouver Sahin F,ÖZEN P,vural f,Kamer S,ARGIN M,Anacak Y,HEKİMGİL M,SAYDAM G Retrospective analysis of seven patients with adult-onset langerhans cell histiocytosis syndromes: A single center experience. Turkish Journal of Hematology. 2007; 24(2): 75 - 79.
IEEE Sahin F,ÖZEN P,vural f,Kamer S,ARGIN M,Anacak Y,HEKİMGİL M,SAYDAM G "Retrospective analysis of seven patients with adult-onset langerhans cell histiocytosis syndromes: A single center experience." Turkish Journal of Hematology, 24, ss.75 - 79, 2007.
ISNAD Sahin, Fahri vd. "Retrospective analysis of seven patients with adult-onset langerhans cell histiocytosis syndromes: A single center experience". Turkish Journal of Hematology 24/2 (2007), 75-79.