Langerhans Hücreli Histiositoz: Tek Merkez Deneyimi

Yıl 2022, , 311 - 316, 27.12.2022

Burçak Kurucu , Şule Yeşil , Ceyhun Bozkurt , Nazmiye Yuksek , Ali Fettah , Gürses Şahin

https://doi.org/10.29058/mjwbs.1094748

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

• 1.Emile JF et al. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood. 2016; 127: 2672-2681.
• 2.Jezierska, M et al. Langerhans cell histiocytosis in children-a disease with many faces. Recent advances in pathogenesis, diagnostic examinations and treatment. Advances in Dermatology and Allergology/Postȩpy Dermatologii i Alergologii. 2018; 35(1): 6.
• 3.Guyot-Goubin A et al. Descriptive epidemiology of childhood Langerhans cell histiocytosis in France, 2000–2004. Pediatr Blood Cancer. 2008; 51:71-75.
• 4.Stålemark H et al. Incidence of Langerhans cell histiocytosis in children: A population‑based study. Pediatr Blood Cancer. 2008; 51: 76‑81.
• 5.Papo M et al. Systemic histiocytosis (Langerhans cell histiocytosis, Erdheim-Chester disease, Destombes-Rosai-Dorfman disease): from oncogenic mutations to inflammatory disorders. Curr Oncol Rep. 2019; 21: 62.
• 6.Haupt R et al. Langerhans cell histiocytosis (LCH): guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years. Pediatr Blood Cancer. 2013; 60: 175–184.
• 7.Ronceray L et al. Pulmonary Involvement in Pediatric-Onset Multisystem Langerhans Cell Histiocytosis: Effect on Course and Outcome. J Pediatrics. 2012; 161: 129–133.
• 8. Khung S et al. Skeletal involvement in Langerhans cell histiocytosis. Insights Imaging. 2013; 4: 569-579.
• 9. Morimoto A et al. Recent advances in Langerhans histiocytosis. Pediatr Int. 2014; 56: 451-461.
• 10.Filipovich A et al. Histiocytic disorders: recent insights into pathophysiology and practical guidelines. Biol Blood Marrow Transplant. 2010; 16: 82-89.
• 11.Fahrner B et al. Long-term outcome of hypothalamic pituitary tumors in Langerhans cell histiocytosis. Pediatr Blood Cancer. 2012; 58: 606-610.
• 12.Grois N et al. Central nervous system disease in Langerhans histiocytosis. J Pediatr. 2010; 156: 873-881.
• 13.Yi X et al. Liver involvement of Langerhans’ cell histiocytosis in children. Int J Clin Exp Med. 2015; 8: 7098-7106.
• 14.Minkov M et al. Bone marrow assessment in Langerhans histiocytosis. Pediatr Blood Cancer. 2007; 49: 694-698.
• 15.Grana N. Langerhans cell histiocytosis. Cancer Control. 2014; 21: 328-334.
• 16.Edelweiss M et al. Lymph node involvement by Langerhans cell histiocytosis: a clinicopathologic and histochemical study of 20 cases. Hum Pathol. 2007; 38: 1463-1469.
• 17.Braier J et al. Isolated pulmonary Langerhans cell histiocytosis presenting with recurrent pneumothorax. Pediatr Blood Cancer. 2007; 48: 241-244.
• 18.Odame I et al. Pulmonary Langerhans cell histiocytosis: a variable disease in childhood. Pediatr Blood Cancer. 2006; 47: 889-893.
• 19.Lin CH et al. Langerhans cell histiocytosis with thyroid and lung involvement in a child: a case report. J Pediatr Hematol Oncol. 2010; 32: 309-311.
• 20. Goyal R et al. Langerhans histiocytosis infiltration into pancreas and kidney. Pediatr Blood Cancer. 2007; 49: 748-750.
• 21.Nelson BL. Langerhans cell histiocytosis of the temporal bone. Head and Neck Pathology. 2008; 2(2): 97–98.
• 22.Nicollas R, et al. Head and neck manifestation and prognosis of Langerhans’ cell histiocytosis in children. Int. J. Pediatr. Otorhinolaryngol. 2010;74(6): 669–673.
• 23.Allen CE et al. How I treat Langerhans cell histiocytosis. Blood. 2015;126: 26‑35.
• 24.Grois N et al. Risk factors for diabetes insipidus in Langerhans histiocytosis. Pediatr Blood Cancer. 2006; 46: 228-233.
• 25.Yağci B et al. Langerhans cell histiocytosis: retrospective analysis of 217 cases in a single center. Pediatr Hematol Oncol. 2008; 25: 399-408.
• 26.Minkov M. Multisystem Langerhans cell histiocytosis in children. Current treatment and future directions. Pediatr Drugs. 2011; 13: 75-86.
• 27.Minkov M et al. Langerhans cell histiocytosis-Histiocyte Society Evaluation and Treatment Guidelines, Protocol, April. 2009.
• 28.Weitzman S et al. 2’-Chlorodeoxyadenosine (2-CdA) as Salvage Therapy for Langerhans Cell Histiocytosis (LCH). Results of the LCH-S-98 Protocol of the Histiocyte Society. Pediatr Blood Cancer. 2009; 53:1271–1276.
• 29.Grana N. Langerhans cell histiocytosis. Cancer Contr. 2014; 21: 328‑334.
• 30.Papadopoulou M et al. The multiple faces of Langerhans cell histiocytosis in childhood: a gentle reminder. Mol Clin Oncol. 2018; 8: 489–492.