Serhat ÇELİK, Zeynep Tuğba GÜVEN, Onur AŞIK, Muzaffer KEKLİK, Leylagül KAYNAR, Bülent ESER, Mustafa ÇETİN, Ali ÜNAL

Adult Onset Langerhans Cell Histiocytosis: A Single Center Experience

Aim: Langerhans cell histiocytosis (LCH) is a proliferative disease caused by abnormal proliferation of histiocytes in the bone marrow dendritic cell structure. LCH is very rare in adults and its incidence is 1-2 cases per million. Therefore, there is still no clear management plan in adults. It was aimed to evaluate the very rare adult LCH patients. Materials and Methods: Ten LCH patients who were followed up at Erciyes University Hematology Department between January 2010 and January 2020 were included. Results: Eight (80%) of them were male and 2 (20%) were female. The median age of the patients was 34.5 ± 8.4 (23-52) years. Although the most common involvement at the time of diagnosis was bone in 7 patients (70%) and lung in 3 patients (30%); pituitary, tympanic membrane, liver and brain involvement were also observed. Three (30%) patients had single system involvement and 7 (70%) had multisystem. Four (40%) patients had relapsed and all had multisystemic involvement. The BRAF V600E mutation could be performed in 3 patients. It was negative in two patients and positive in 1 patient. All patients still have remission. Conclusion: Unlike children, LCH has a better course in adults. The most important approach is to determine single or multisystem involvement. In our patients with single system involvement, we obtained response with corticosteroid and surgery alone. We have observed that vinblastin plus metilprednizolon treatment is a good option for multisystemic involvement. In relapsed patients, we obtained a significant response with clofarabine.

Keywords:

Adult, BRAF V600E mutation, clofarabin, histiocytosis, langerhans cell, Radiotherapy, Treatment, vinblastin plus methylprednisolone Adult, BRAF V600E mutation, clofarabin, histiocytosis, langerhans cell, Radiotherapy, Treatment, vinblastin plus methylprednisolone,

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Referans1. AKDEMİR O, ÇOLAK A. Langerhans hücresi histiositozisi. Turkiye Klinikleri Journal of Surgical Medical Sciences. 2007;3(51):1-4.
Referans2. Alston RD, Tatevossian R, McNally RJ, Kelsey A, Birch JM, Eden TO. Incidence and survival of childhood Langerhans cell histiocytosis in Northwest England from 1954 to 1998. Pediatric blood & cancer. 2007;48(5):555-60.
Referans3. McClain K. Treatment of langerhans cell histiocytosis. UpToDate. Waltham, MA 2013.
Referans4. Weitzman S, Braier J, Donadieu J, Egeler RM, Grois N, Ladisch 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. Pediatric blood & cancer. 2009;53(7):1271-6.
Referans5. Gadner H, Minkov M, Grois N, Pötschger U, Thiem E, Arico M, et al. Therapy prolongation improves outcome in multisystem Langerhans cell histiocytosis. Blood, The Journal of the American Society of Hematology. 2013;121(25):5006-14.
Referans6. Allen CE, Flores R, Rauch R, Dauser R, Murray JC, Puccetti D, et al. Neurodegenerative central nervous system Langerhans cell histiocytosis and coincident hydrocephalus treated with vincristine/cytosine arabinoside. Pediatric blood & cancer. 2010;54(3):416-23.
Referans7. Simko SJ, Tran HD, Jones J, Bilgi M, Beaupin LK, Coulter D, et al. Clofarabine salvage therapy in refractory multifocal histiocytic disorders, including Langerhans cell histiocytosis, juvenile xanthogranuloma and Rosai–Dorfman disease. Pediatric blood & cancer. 2014;61(3):479-87.
Referans8. McClain KL, Picarsic J, Chakraborty R, Zinn D, Lin H, Abhyankar H, et al. CNS Langerhans cell histiocytosis: Common hematopoietic origin for LCH‐associated neurodegeneration and mass lesions. Cancer. 2018;124(12):2607-20.
Referans9. Sahin F, Ozen K, Vural F, Kamer S, Argin M, Anacak Y. Retrospective analysis of seven patients with adult-onset Langerhans cell histiocytosis syndromes: A single center experience. Turk J Hematol. 2007;24:75-9.
Referans10. Grois N, Pötschger U, Prosch H, Minkov M, Arico M, Braier J, et al. Risk factors for diabetes insipidus in langerhans cell histiocytosis. Pediatric blood & cancer. 2006;46(2):228-33.
Referans11. Prosch H, Grois N, Prayer D, Waldhauser F, Steiner M, Minkov M, et al. Central diabetes insipidus as presenting symptom of Langerhans cell histiocytosis. Pediatric blood & cancer. 2004;43(5):594-9.
Referans12. Di Iorgi N, Allegri AEM, Napoli F, Calcagno A, Calandra E, Fratangeli N, et al. Central diabetes insipidus in children and young adults: etiological diagnosis and long-term outcome of idiopathic cases. The Journal of Clinical Endocrinology & Metabolism. 2014;99(4):1264-72.
Referans13. Lindahl L, Fenger‐Grøn M, Iversen L. Topical nitrogen mustard therapy in patients with Langerhans cell histiocytosis. British Journal of Dermatology. 2012;166(3):642-5.
Referans14. McClain KL, Kozinetz CA. A phase II trial using thalidomide for Langerhans cell histiocytosis. Pediatric blood & cancer. 2007;48(1):44-9.
Referans15. Cantu MA, Lupo PJ, Bilgi M, Hicks MJ, Allen CE, McClain KL. Optimal therapy for adults with Langerhans cell histiocytosis bone lesions. PloS one. 2012;7(8).
Referans16. Duan M-H, Han X, Li J, Zhang W, Zhu T-N, Han B, et al. Comparison of vindesine and prednisone and cyclophosphamide, etoposide, vindesine, and prednisone as first-line treatment for adult Langerhans cell histiocytosis: A single-center retrospective study. Leukemia research. 2016;42:43-6.
Referans17. Minkov M, Steiner M, Pötschger U, Aricò M, Braier J, Donadieu J, et al. Reactivations in multisystem Langerhans cell histiocytosis: data of the international LCH registry. The Journal of pediatrics. 2008;153(5):700-5. e2.
Referans18. Abraham A, Alsultan A, Jeng M, Rodriguez‐Galindo C, Campbell PK. Clofarabine salvage therapy for refractory high‐risk langerhans cell histiocytosis. Pediatric blood & cancer. 2013;60(6):E19-E22.
Referans19. Willis B, Ablin A, Weinberg V, Zoger S, Wara WM, Matthay KK. Disease course and late sequelae of Langerhans' cell histiocytosis: 25-year experience at the University of California, San Francisco. Journal of clinical oncology. 1996;14(7):2073-82.
Referans20. Sivendran S, Harvey H, Lipton A, Drabick J. Treatment of Langerhans cell histiocytosis bone lesions with zoledronic acid: a case series. International journal of hematology. 2011;93(6):782-6.
Referans21. Heritier S, Emile J-F, Barkaoui M-A, Thomas C, Fraitag S, Boudjemaa S, et al. BRAF mutation correlates with high-risk Langerhans cell histiocytosis and increased resistance to first-line therapy. Journal of clinical oncology. 2016;34(25):3023.
Referans22. Váradi Z, Bánusz R, Csomor J, Kállay K, Varga E, Kertész G, et al. Effective BRAF inhibitor vemurafenib therapy in a 2-year-old patient with sequentially diagnosed Langerhans cell histiocytosis and Erdheim–Chester disease. OncoTargets and therapy. 2017;10:521.
Referans23. Héritier S, Jehanne M, Leverger G, Emile J-F, Alvarez J-C, Haroche J, et al. Vemurafenib use in an infant for high-risk Langerhans cell histiocytosis. JAMA oncology. 2015;1(6):836-8.
Referans24. Diamond EL, Durham BH, Ulaner GA, Drill E, Buthorn J, Ki M, et al. Efficacy of MEK inhibition in patients with histiocytic neoplasms. Nature. 2019;567(7749):521-4.