ÇOKLU ORGAN TUTULUMU OLAN LANGERHANS HÜCRELİ HİSTİOSİTOZİS; OLGU SUNUMU

Year 2021, Volume: 1 Issue: 2 - October 2021, 2 - 8, 26.12.2021

Aygül Güzel Nurhan Köksal Fatma Zeynep Özen Ayşenur Arlı

https://doi.org/10.54270/atljm.2021.6

Abstract

Langerhans Hücreli Histiositozis (LHH) sebebi tam olarak bilinmeyen nadir görülen bir hastalıktır. Epitel yüzeylerde bulunan, dendritik hücrelerin alt tipi olan monoklonal langerhans hücrelerinin anormal çoğalması ile karakterizedir. Sıklıkla çocuk yaş grubunda gözlenir ancak nadiren erişkin yaş grubunda da gözlenebilmektedir. LHH sıklıkla tek organ tutulumu ile seyreder fakat akciğer, kemik, hipofiz bezi, deri, karaciğer gibi çok sayıda organ tutulumu da gözlenebilir. Organ tutulumlarına göre klinik seyirde farklılıklar ortaya çıkmaktadır. LHH nadir görülen bir hastalık olması ve hastamızda akciğer, karaciğer ve yaygın cilt tutulumu olması nedeniyle sunulmuştur.

Keywords

Langerhans Hücreli Histiositozis, deri, çoklu organ tutulumu, sklerozankolanjit

References

• Mello RAF, Tanos JW, Mello MBN, Marchiorive E. Multisystemic Langerhans Cell Histiocytosis with advanced lung involvement. Radiology Case. 2012; 6(11):22-28.
• Choi JE, Lee HR, Ohn JH, Moon MK, Park J, Lee SJ et all. Adult Multisystem Langerhans Cell Histiocytosis Presenting with Central Diabetes Insipidus Successfully Treated with Chemotherapy. Endocrinol Metab (Seoul). 2014;29(3):394-9.
• Kobayashi M, Tojo A. Langerhans cell histiocytosis inadults:Advances inpathophysiology and treatment. Cancer Science. 2018;109:3707–13.
• Aricò M, Girschikofsky M, Généreau T, Klersy C, McClain K, Grois N et all. Langerhans cell histiocytosis in adults. Report from the International Registry of the Histiocyte Society. Eur J Cancer. 2003;39(16):2341-8.
• Lian C, Lu Y, Shen S. Langerhans cell histiocytosisi in adults: a case report and review of the literatür. Oncotarget 2016;7(14):18678-83.
• Willman CL. Detection of clonal histiocytes in Langerhans cell histiocytosis: biology and clinical significance. Br J Cancer Suppl. 1994;23:S29-33.
• Gillott M, Flemming B, Ravenel JG. Imaging of cystic lung disease. Semin Roentgenol. 2015;50(1):23-30. Epub 2014 Apr 13.
• Haupt R, Minkov M, Astigarraga I, Schäfer E, Nanduri V, Jubran R, et all. 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
• Scanzi J, Goutte M, Teilhet C, Abergel A, When should we consider transplantation in adult patients with sclerosing cholangitis due to multisystem Langerhans’ cell histiocytosis? Digestive and Liver Disease 47 (2015) 175–179.
• Macras P, Kaltsas G. Langerhans cell histiocytosis and pituitary function. Endocrine. 2015;48(3):728-9. Epub 2015 Jan 1.
• Edelbroek JR, Vermeer MH, Jansen PM, Stoof TJ, van der Linden MM, Horváth B, et all. Langerhans cell histiocytosis first presenting in the skin in adults: frequent association with a second haematological malignancy. British Association of Dermatologists 2012; 167:1287–1294
• Simko SJ, Garmezy B, Abhyankar H, Lupo PJ, Chakraborty R, Lim KPH et all. Differentiating skin-limited and multisystem Langerhans cell histiocytosis. J Pediatr. 2014;165(5): 990–996.