Langerhans Hücreli Histiositoz: Tek Merkez Deneyimi

Öz

Amaç: Langerhans hücreli histiositoz (LHH), tüm organ ve sistemleri etkileyebilen ve çok çeşitli klinik bulgulara neden olabilen nadir bir hastalıktır. Tedavi ve prognoz organ tutulumu bölgesine ve riskine göre değişkenlik gösterir. Bu çalışmada, kliniğimizde LHH tanısıyla takip edilen hastaları değerlendirmeyi amaçladık. Gereç ve Yöntemler: Çalışmada Ocak 2000 ile Aralık 2019 arasında LCH tanısı alan 24 hastanın verileri geriye dönük olarak incelendi. Hastalar tanı anındaki yaş, cinsiyet, başvuru semptomları, organ tutulumu, tedaviler, takip süresi ve tetavi yanıtları açısından değerlendirildi. Bulgular: Hastaların tanı yaşı ortalaması 4,7±4,6 (0,6-16,6) yıl ve ortalama takip süresi 91,6±67,8 (12,1-240) ay idi. Tanı anındaki en sık görülen bulgu cilt lezyonları iken, en sık görülen organ tutulumları kemik ve ciltti. Bunu sırasıyla hipofiz, karaciğer, lenf nodu, akciğer, dalak, periodontal bölge, tiroid, orbital bölge, mastoid, pankreas ve kemik iliği izledi. Hastalara risk gruplarına göre LCH-III çalışma protokolüne uygun olarak tedavi uygulandı. Progresyon gösteren 6 hastanın 5’ine kladribin tedavisi, 1 hastaya hematopoetik kök hücre nakli (HKHN) uygulandı ve hepsinde tam yanıt elde edildi. Sonuç: LHH’de tutulan organ ve sistemlere, hastalığın yaygınlığına göre kemoterapi, radyoterapi ve cerrahi tedavi seçenekleri farklı kombinasyonlarda uygulanmaktadır. Yanıtsızlık veya progresyon durumlarında bizim serimizde olduğu gibi kladribin ve HKHN tedavileri başarılı bir şekilde uygulanmaktadır.

Anahtar Kelimeler

• Langerhans hücreli histiositoz
• Kladribin
• Hematopoetik kök hücre nakli

Langerhans Cell Histiocytosis: A Single Center Experience

Öz

Aim: Langerhans cell histiocytosis (LCH) is a rare disease that can affect all organs and systems and cause a wide variety of clinical findings. Treatment and prognosis vary according to site and risk of organ involvement. In this study, we aimed to evaluate the patients who were followed up with diagnosis of LCH in our clinic. Materials and methods: In the study, data of 24 patients diagnosed with LCH between January 2000 and December 2019 were retrospectively analyzed. The patients were evaluated in terms of age at diagnosis, gender, initial symptoms, organ involvement, treatments, follow-up period, and outcome. Results: The mean age at diagnosis was 4.7±4.6 (0.6-16.6) years, and the mean follow-up time was 91.6±67.8 (12.1-240) months. While the most common symptom at the time of diagnosis was skin lesions, the most common organ involvements were bone and skin. This was followed by the pituitary, liver, lymph node, lung, spleen, periodontal region, thyroid, orbital region, mastoid, pancreas, and bone marrow, respectively. Patients were treated in accordance with the LCH-III study protocol according to risk groups. Cladribine treatment was applied to 5 of 6 patients who progressed, and hematopoietic stem cell transplantation (HSCT) was applied to 1 patient, and complete response was obtained in all of them. Conclusion: Depending on the organs and systems involved in LCH and the extent of the disease; chemotherapy, radiotherapy and surgical treatment options are applied in different combinations. In cases of unresponsiveness or progression, cladribine and HSCT treatments are successfully applied, as in our series.

Anahtar Kelimeler

• Langerhans cell histiocytosis
• Cladribine
• Hematopoietic stem cell transplantation

Kaynakça

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