Adult Onset Langerhans Cell Histiocytosis

Öz

Langerhans cell histiocytosis (LCH) is a rare disease characterized by clonal and neoplastic proliferation of Langerhans cells. It is most commonly seen in children but can be seen at any age. Clinical findings depend on the organ system involved. The diagnosis of LCH is made histopathologically. The treatment is planned according to the organ system of the disease, the prevalence of the lesions and the presence of specific organ dysfunction. We aimed to discuss the clinical, radiological and histapatological findings of a 33-year-old man with femoral involvement at adulthood and discuss the current literature.

Anahtar Kelimeler

• Langerhans cells,histiocytosis,adult onset

Erişkin Başlangıçlı Langerhans Hücreli Histiyositoz

Öz

Langerhans hücreli histiyositoz (LHH), Langerhans hücrelerinin klonal ve neoplastik proliferasyonu ile karakterize nadir bir hastalıktır. En sık çocuklarda tanı almakla beraber, her yaşta görülebilir. Klinik bulgular, tutulan organ sistemine göre değişmektedir. LHH'nin kesin tanısı histopatolojik olarak konulmaktadır. LHH’un tedavisi, hastalığın bulunduğu organ sistemine ve lezyonların yaygınlığına göre planlanmaktadır. Biz burada erişkin yaş başlangıçlı, femur tutulumu olan 33 yaşında erkek hastanın klinik, radyolojik ve histapatolojik bulguları sunularak güncel literatür eşliğinde tartışmayı amaçladık.

Anahtar Kelimeler

• histiyositozis,erişkin başlangıç,Langerhans hücreleri,erişkin başlangıç

Kaynakça

1. Scolozzi, P., Lombardi, T., Monnier, P., & Jaques, B. (2004). Multisystem Langerhans’ cell histiocytosis (Hand-Schüller-Christian disease) in an adult: a case report and review of the literature. European Archives of Oto-Rhino-Laryngology and Head & Neck, 261(6), 326-330.
2. Tun, K., Türkoğlu, Ö. F., Okutan, Ö., Yeşilkılıç, Ş., Beşkonaklı, E., & Özen, Ö. (2005). Eosinophilic Granuloma in the young adult patient: A Case Report. Journal of Neurological Sciences (Turkish), 22(4), 421-424.
3. Lipton JM. Histiocytic disorders. In:Hoofman R, Benz EJ, Shattil SJ, et al. editors. Basic Principals and Practice. 3rd ed. New York: Churchill Livingstone, 2000:783-93.
4. Akdemir, O., & Çolak, A. (2007). Langerhans hücresi histiositozisi. Turkiye Klinikleri Journal of Surgical Medical Sciences, 3(51), 1-4.
5. Scappaticci, S., Danesino, C., Rossi, E., Klersy, C., Fiori, G. M., Clementi, R., ... & AIEOP‐Istiocitosi Group. (2000). Cytogenetic abnormalities in PHA‐stimulated lymphocytes from patients with Langerhans cell histiocytosis. British journal of haematology, 111(1), 258-262.
6. McClain, K., Jin, H., Gresik, V., & Favara, B. (1994). Langerhans cell histiocytosis: lack of a viral etiology. American journal of hematology, 47(1), 16-20.
7. Jordan, M. B., McClain, K. L., Yan, X., Hicks, J., & Jaffe, R. (2005). Anti‐CD52 antibody, alemtuzumab, binds to Langerhans cells in Langerhans cell histiocytosis. Pediatric blood & cancer, 44(3), 251-254.
8. Betts, D. R., Leibundgut, K. E., Feldges, A., Plüss, H. J., & Niggli, F. K. (1998). Cytogenetic abnormalities in Langerhans cell histiocytosis. British journal of cancer, 77(4), 552.

9. Lieberman, P. H., Jones, C. R., Steinman, R. M., Erlandson, R. A., Smith, J., Gee, T., ... & Gangi, M. D. (1996). Langerhans cell (eosinophilic) granulomatosis: a clinicopathologic study encompassing 50 years. The American journal of surgical pathology, 20(5), 519-552.
10. Günaldı, M., Duman, B. B., Afşar, Ç. U., Günaldı, Ö., & Şahin, B. Temporal kemik yerleşimli langerhans hücreli histiositosis: Bir olgu sunumu.
11. Hirata, Y., Schulz, M., Altenmüller, E., Elbert, T., & Pantev, C. (2004). Sensory mapping of lip representation in brass musicians with embouchure dystonia. Neuroreport, 15(5), 815-818.
12. Çil T, Gökalp D, Tuzcu A, Işıkdoğan A, Bahçeci M. Yetişkin Langerhans hücreli histiyositoz. Turkiye Klinikleri J MedSci 2007;27: 633- 5.
13. Serdar, Z. A., Gizenti, S., Yaşar, Ş., Gönenç, I., & Güneş, P. (2012). Adult Onset Langerhans' Cell Histiocytosis: A Case Report. Marmara Medical Journa l, 25, 96-99.