Erişkin Başlangıçlı Langerhans Hücreli Histiositozis Tanılı Olguların Retrospektif Değerlendirilmesi: 14 Olguluk Tek Merkez Deneyimi

Öz

Amaç: Langerhans hücreli histiyositoz LHH , nedeni bilinmeyen, atipik histiyositlerin lokal veya yaygın olarak deri, kemik, akciğer, karaciğer, lenf nodları, mukokutanoz dokular ve endokrin organlar gibi çeşitli dokularda birikmesi sonucunda hasara sebep olan bir hastalık grubudur. Biz bu çalışmada merkezimizde tanısı konulan ve takip edilen 14 LHH olgusunun demografik ve klinik özelliklerini sunmak istedik.Gereç ve Yöntemler: Çalışmada 01-Mart-2011/30-Eylül-2019 tarihleri arasında Sağlık Bilimleri Üniversitesi Ankara Dışkapı Yıldırım Beyazıt Eğitim ve Araştırma Hastanesi Hematoloji Bölümünde LHH tanısı ile izlenen 14 hasta 7 kadın, 7 erkek retrospektif olarak değerlendirildi.Bulgular: Olguların 5’inde kemik tutulumu, 5’inde lenf nodu, 3’ünde cilt, 2 hastada beyin, 1 hastada karaciğer, 1 hastada akciğer, 1 hastada uterus, 1 hastada parotis, 1 hastada kolon, 1 hastada tiroid, tutulumu mevcuttu. Toplam takip süresi 103 ay sonunda tüm hastalar hayattadır. Tek lezyonu olan hastaların tamamı cerrahi olarak tedavi edilirken, multisistemik tutulumu olan 3 hasta cladribine içeren kemoterapi aldı.Sonuç: Merkezimizde 8 yıllık süre içerisinde erişkin yaş grubunda LHH tanısı alan sadece 14 hasta vardı. Tedavi planı hastanın klinik şikayetlerine ve kitlenin bası bulgularına göre belirlenir. Hastalar tedavisiz izlemle takip edilebildiği gibi, acil tedavi başlanacak kadar hızlı ve kötü seyirli prognoza sahip olabilirler. Değişken klinik seyir, hastalığın doğası gereği midir yoksa, az ve etkin olmayan ve zamanında verilmeyen tedaviler prognozu olumsuz mu etkilemektedir? Sadece lokal uygulanan tedaviler başarılı mıdır? gibi sorulara cevap bulmak amacıyla daha büyük hasta serileri ile yapılacak çalışmalara ihtiyaç vardır

Anahtar Kelimeler

• Langerhans hücreli histiositoz, Erişkin başlangıçlı, Tek merkez deneyimi

A Retrospective Evaluation of Cases with Adult-Onset Langerhans Cell Histiocytosis: A Single-Center Experience of 14 Cases

Abstract

Objective: Langerhans cell histiocytosis LHH is a disease group of unknown etiology that causes tissue injury as a result of the local or systemic accumulation of atypical histiocytes in various tissues such as skin, bone, lungs, liver, lymph nodes, mucocutaneous tissues, and endocrine organs. We aimed to report the demographic and clinical properties of 14 LLH cases diagnosed and followed at our center.Material and Methods: This study retrospectively enrolled 14 patients 7 females, 7 males having a diagnosis of LLH who were followed up at the Health Sciences University Ankara Dışkapı Yıldırım Beyazıt Training and Research Hospital, Department of Hematology between 01-March-2011 and 30-September-2019.Results: Five patients had bone involvement; 5 had lymph node, 3 had skin, 2 had brain, 1 had liver, 1 had lung, 1 had uterine, 1 had parotid, 1 had colon, and 1 had thyroid involvement. At the end of a total follow-up period of 103 months, all patients are alive. All patients with single organ involvement were treated surgically while 3 patients with multisystemic involvement received chemotherapy containing cladribine.Conclusion: There were only 14 adults diagnosed with LHH at our center over a period of 8 years. Treatment is tailored to patient’s symptoms and compressive signs of a mass. While patients may be managed conservatively, without treatment, they may have a rapid and grave prognosis that requires commencing urgent therapy. Is a variable clinical course a characteristic feature of the disease or do inadequate and ineffective treatments unfavorably affect prognosis? Are solely local treatments effective? There is a need for studies with larger patient series

Keywords

• Langerhans Cell Histiocytosis, Adult-onset, Single-Center Experience

Kaynakça

1. Jaffe R. The diagnostic histopathology of Langerhans cell histiocytosis. In: Weitzman S, Egeler RM, eds. Histiocytic Disorders of Children and Adults. Basic Science, Clinical Features, and Therapy, Cambridge: Cambridge University Press, 2005; p14.
2. Gadner H, Grois N, Pötschger U, Minkov M, Aricò M, Braier J, Broadbent V, Donadieu J, Henter JI, McCarter R, Ladisch S. Improved outcome in multisystem Langerhans cell histiocytosis is associated with therapy intensification. Blood 2007; 111:2556-62.
3. Guyot-Goubin A, Donadieu J, Barkaoui M, Bellec S, Thomas C, Clavel J. Descriptive epidemiology of childhood Langerhans cell histiocytosis in France, 2000- 2004. Pediatr Blood Cancer 2008; 51:71-5.
4. Lipton JM. Histiocytic disorders. In:Hoofman R, Benz EJ, Shattil SJ, Furie B, Cohen HJ, Silberstein LE, McGlave P, eds. Basic Principals and Practice. 3rd ed. New York: Churchill Livingstone, 2000:783-93.
5. Arico M, Girschikofsky M, Genereau T, Klersy C, McClain K, Grois N, Emile JF, Lukina E, De Juli E, Danesino C. Langerhans cell histiocytosis in adults. Report from the international registry of the Histiocyte Society. Eur J Cancer 2003; 39:2341-8.
6. Yağci B, Varan A, Cağlar M, Söylemezoğlu F, Sungur A, Orhan D, Yalçin B, Akyüz C, Kutluk T, Büyükpamukçu M. Langerhans cell histiocytosis: Retrospective analysis of 217 cases in a single center. Pediatr Hematol Oncol 2008; 25:399-408.
7. Şahin F, Özen KP, Vural F, Kamer S, Argın M, Anacak Y, Hekimgil M, Saydam G. Retrospective analysis of seven patients with adult-onset langerhans cell histiocytosis syndromes: A single center experience. Turk J Haematol 2007; 24(2):75-9.
8. Emile JF, Abla O, Fraitag S, Horne A, Haroche J, Donadieu J, Requena-Caballero L, Jordan MB, Abdel- Wahab O, Allen CE, Charlotte F, Diamond EL, Egeler RM, Fischer A, Herrera JG, Henter JI, Janku F, Merad M, Picarsic J, Rodriguez-Galindo C, Rollins BJ, Tazi A, Vassallo R, Weiss LM. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood 2016; 127(22):2672-81.

9. Hegemann MV, Schreml S. Multisystemic Langerhans cell histiocytosis in an adult. JAAD Case Rep 2017; 3(2):162-4.
10. Takei Y, Ueshima C, Kataoka TR, Hirata M, Sugimoto A, Rokutan-Kurata M, Moriyoshi K, Ono K, Murakami I, Iwamoto S, Haga H. Killer cell immunoglobulin-like receptor 2DL4 is expressed in and suppresses the cell growth of Langerhans cell histiocytosis. Oncotarget 2017; 8(23):36964-72.