#PAGE_PARAMS# #ADS_HEAD_SCRIPTS# #MICRODATA#
CS
proLékaře.cz / Journals / Transfusion and Haematology Today / 2020 - 2

Treatment of Langerhans cell histiocytosis in adults

Czech version

Authors: Z. Král 1;  Z. Adam 1;  M. Doubková 2;  M. Krejčí 1;  T. Horvát 3;  M. Doubek 1;  L. Pour 1;  Z. Řehák 4,5;  R. Koukalová 4
Authors‘ workplace: Interní hematologická a onkologická klinika LF MU a FN Brno 1;  Klinika nemocní plicních a TBC LF MU a FN Brno 2;  Chirurgická klinika LF MU a FN Brno 3;  Oddělení nukleární medicíny, Masarykův onkologický ústav, Brno 4;  Regionální centrum aplikované molekulární onkologie (RECAMO), Masarykův onkologický ústav, Brno 5
Published in: Transfuze Hematol. dnes,26, 2020, No. 2, p. 101-112.
Category: Review/Educational Papers

Overview

Langerhans cell histiocytosis has diverse clinical manifestations and the aggressiveness of its course varies. As in the case of other malignant diseases, involvement of other organs must be determined once the diagnosis has been made.  Local treatment may suffice in the case of a single lesion (surgery, instillation of methylprednisolone within the lesion). Skin involvement responds to phototherapy (PUVA or electron beam irradiation). Remissions following the administration of thalidomide or methotrexate have been described in cases of isolated skin involvement.  In adults, 2-cholorodeoxyadenosine (cladribine) in monotherapy and less often in combination with cytosine-arabinoside is the treatment of choice in the case of multi-system involvement. Extremely aggressive forms may be treated with combined chemotherapy regimens that include etoposide as in the case of lymphomas. In resistant cases, patients may be referred to transplant centres and undergo high-dose chemotherapy with autologous or allogeneic stem cell transplantation. Patients with the BRAF V600E gene mutation have been shown to respond to vemurafenib or dabrafenib, which are registered in the Czech Republic for the treatment of disseminated melanoma. On demonstrating this mutation and documenting resistance to prior therapy, it would be possible to request reimbursement of these agents from the health insurance company. 

Keywords:

Langerhans cell histiocytosis – 2-cholorodeoxyadenosine (cladribine) – vemurafenib – dabrafenib

Sources
  1. Adam Z, Ježová M, Nebeský T, et al. Klinické obrazy histiocytózy z Langerhansových buněk v dospělosti. Transfuze Hematol Dnes. 2019;25(3):219–228.
  2. Arico M. Langerhans cell histiocytosis in adults. Report from the International Registry of the Histiocyte Society. Eur J Cancer. 2003;39(16):2341–2348.
  3. Girschikofsky M, Arico M, Castilo D, et al. Management of adult patients with Langerhans cell histiocytosis, recommendation from an expert panel on behalf of Euro-Histo Net. Orphanet J Rare Dis. 2013; 8:72–78.
  4. Badalian-Very G, Vergilio JA, Degar BA, et al. Recurrent BRAF mutations in Langerhans cell histiocytosis. Blood. 2010;116(11):1919–1923.
  5. Swerdlow SH, Campo E, Harris NL, et al. WHO classification of tumours of haematopoietic and lymphoid tissues. International Agency for Research on Cancer (IARC), 2008; 439 pages.
  6. Lorillon G, Tazi A. How I manage pulmonary Langerhans cell histiocytosis. Eur Respir Rev. 2017;26(145).
  7. Doubková M, Tomíšková M, Skřičková J. Plicní histiocytóza z Langerhansových buněk – nemoc kuřáků. Stud Pneumol Phtiseol. 2014;74(5):158–161.
  8. Tazi A, Marc K, Dominique S, et al. Serial CT and lung function testing in pulmonary Langerhans cell histiocytosis. Eur Respir J. 2012;40(4):905–912.
  9. Auerswald U, Barth J, Magnussen H. Value of CD-1 positive cells in bronchoalveolar lavage fluid for the diagnosis of pulmonary histiocytosis X. Lung. 1991;169(6):305–309.
  10. Lommatzsch M, Bratke K, Stoll P, et al. Bronchoalveolar lavage for the diagnosis of pulmonary Langerhans cell histiocytosis. Respir Med. 2016; 119:168–174.
  11. Yasko AW, Fanning CV, Avala AG, et al. Percutaneous techniques for the diagnosis and treatment of localized Langerhans-cell histiocytosis (eosinophilic granuloma of bone). J Bone Joint Surg Am. 1998;80(2):219–228.
  12. Sakai H, Ibe M, Takahashi H, et al. Satisfactory remission achieved by PUVA therapy in Langerhans cell hisiocytosis in an elderly patient. J Dermatol. 1996;23(1):42–46.
  13. Imafuku S, Shibata S, Tashiro A, et al. Cutaneous Langerhans cell histiocytosis in an elderly man successfully treated with narrowband ultraviolet B. Br J Dermatol. 2007;157(6):1277–1279.
  14. Adam Z, Ježová M, Šlampa P, et al. Histiocytóza z indeterminovaných buněk – vymizení kožní infiltrace po ozáření elektronovým svazkem a aplikace 2-chlorodeoxyadenozinu: kazuistika. Vnitř Lék. 2017;63(4):284–288.
  15. McClain KL, Kozinetz CA. A phase II trial using thalidomide for Langerhans cell histiocytosis. Pediatr Blood Cancer. 2007;48(1):44–49.
  16. Steen AE, Steen KH, Bauer R, et al. Successful treatment of cutaneous Langerhans cell histiocytosis with low-dose methotrexate. Br J Dermatol. 2001;145(1):137–140.
  17. Vassallo R, Harari S, Tazi A. Current understanding and management of pulmonary Langerhans cell histiocytosis. Thorax. 2017;72(10):937–945.
  18. Tazi A, Lorillon G, Haroche J, et al. Vinblastine chemotherapy in adult patients with langerhans cell histiocytosis: a multicenter retrospective study. Orphanet J Rare Dis. 2017;12(1):95.
  19. Lorillon G, Jouenne F, Baroudjian B, et al. Response to trametinib of a pulmonary Langerhans cell histiocytosis harboring a MAP2K1 deletion. Am J Respir Crit Care Med. 2018;198(5):675–678.
  20. Dauriat G, Mal H, Thabut JF, et al. Lung transplantation for pulmonary Langerhans´ cell histiocytosis: a multicenter analysis. Transplantation. 2006;81(5):746–750.
  21. Karampitsakos T, Tzouvelekis A, Chrysikos S, et al. Pulmonary hypertension in patients with interstitial lung disease. Pulm Pharmacol Ther. 2018;50:38–46.
  22. Dimopoulos MA, Theodorakis M, Kostis E, et al. Treatment of Langerhans cell histiocytosis with 2 chlorodeoxyadenosine. Leuk Lymphoma. 1997;25(1–2):187–189.
  23. Donadieu J, Bernard F, van Noesel M for Salvage Group of the Histiocyte Society. Cladribine and cytarabine in refractory multisystem Langerhans cell histiocytosis: results of an international phase 2 study. Blood. 2015;126(12):1415–1423.
  24. Cantu MA, Lupo PJ, Bilgi M, et al. Optimal therapy for adults with Langerhans cell histiocytosis bone lesions. PLoS One. 2012;7(8): e43257.
  25. Derenzini E, Fina MP, Stefoni V, et al. MACOP-B regimen in the treatment of adult Langerhans cell histiocytosis: experience on seven patients. Ann Oncol. 2010;21(6):1173–1178.
  26. Montella L, Merola C, Merola G, et al. Zoledronic acid in treatment of bone lesions by Langerhans cell histiocytosis. J Bone Miner Metab. 2009;27(1):110–113.
  27. Pan Y, Xi R, Wang C, et al. Autologous hematopoietic stem cell transplantation for efficient treatment of multisystem, high-risk, BRAF V600E-negative Langerhans cell histiocytosis. J Int Med Res. 2019;47(9):4522–4529.
  28. Micke O, Seegenschmiedt MH. Consensus guidelines for radiation therapy of benign diseases: a multicenter approach in Germany. Int J Radiat Oncol Biol Phys. 2002;52(2):496–513.
  29. Yeh EA, Greenberg J, Abla O, et al. North American Consortium for Histiocytosis. Evaluation and treatment of Langerhans cell histiocytosis patients with central nervous system abnormalities: Current views and new vistas. Pediatr Blood Cancer. 2018;65(1):e26784.
  30. Novosad O, Skrypets T, Pastushenko Y, et al. MAPK/ERK signal pathway alterations in patients with Langerhans cell histiocytosis. Klin Onkol. 2018;31(2):130–134.
  31. Hyman DM, Puzanov I, Subbiah V, et al. Vemurafenib in multiple nonmelanoma cancers with BRAF V600 mutations. N Engl J Med. 2015;373(8):726–736.
  32. Haroche J, Cohen-Aubart F, Emile JF, et al. Dramatic efficacy of vemurafenib in both multisystemic and refractory Erdheim-Chester disease and Langerhans cell histiocytosis harboring the BRAF V600E mutation. Blood. 2013;121(9):1495–500.
  33. Diamond EL, Subbiah V, Lockhart AC, et al. Vemurafenib for BRAF V600-mutant Erdheim-Chester disease and Langerhans cell histiocytosis: analysis of data from the histology-independent, phase 2, open-label VE-BASKET study. JAMA Oncol. 2018;4(3):384–388.
  34. Heisig A, Sörensen J, Zimmermann SY, et al. Vemurafenib in Langerhans cell histiocytosis: report of a pediatric patient and review of the literature. Oncotarget. 2018;9(31):22236–22240.
  35. Charles J, Beani JC, Fiandrino G, et al. Major response to vemurafenib in patient with severe cutaneous Langerhans cell histiocytosis harboring BRAF V600E mutation. J Am Acad Dermatol. 2014;71(3):e97–e99.
  36. Váradi Z, Bánusz R, Csomor J, et al. Effective BRAF inhibitor vemurafenib therapy in a two-year-old patient with sequentially diagnosed Langerhans cell histiocytosis and Erdheim-Chester disease. Onco Targets Ther. 2017;10:521–526.
  37. Haroche J, Cohen-Aubart F, Emile JF, et al. Vemurafenib as first line therapy in BRAF-mutated Langerhans cell histiocytosis. J Am Acad Dermatol. 2015;73(1):e29–e30.
  38. Donadieu J, Larabi IA, Tardieu M, et al. Vemurafenib for refractory multisystem Langerhans cell histiocytosis in children: an international observational study. J Clin Oncol. 2019;37(31):2857–2865.
  39. Kolenová A, Bubanská E, Špotová A, et al. Cielená liečba závažnej multisystémovej histiocytózy z Langerhansových buniek. Pediatr Prax. 2018;19(1):27–31.
  40. Oneal PA, Kwitkowski V, Luo L, et al. FDA approval summary: vemurafenib for the treatment of patients with Erdheim-Chester disease with the BRAFV600 mutation. Oncologist. 2018;23(12):1520–1524.
  41. Cohen Aubart F, Emile JF, Carrat F, et al. Targeted therapy in 54 patients with Erdheim Chester disease, including follow up after interruption (the LOVE study). Blood. 2017;130:1377–1380.
  42. Al Bayati A, Plate T, Al Bayati M, et al. Dabrafenib and trametinib treatment for Erdheim-Chester disease with brain stem involvement. Mayo Clin Proc Innov Qual Outcomes. 2018;2(3):303–308.
  43. Stewart JR, Murzaku EC, Sode TT, Gordon KA. Cutaneous Langerhans cell histiocytosis with gastrointestinal involvement treated with dabrafenib. JAAD Case Rep. 2017;4(1):95–97.
  44. Szturz P, Řehák Z, Koukalová R. Measuring diffuse metabolic activity on FDG-PET/CT: new method for evaluating Langerhans cell histiocytosis activity in pulmonary parenchyma. Nucl Med Biol. 2012;39(3):429–436.
  45. Adam Z, Řehák Z, Koukalová R, et al. Plicní forma histiocytózy z Langerhansových buněk: hodnocení aktivity nemoci a léčebné odpovědi pomocí PETCT (indexu SUVmax Pulmo/ SUVmax Hepar). Popis vlastních zkušeností a přehled literatury. Vnitř Lék. 2010;56(12):1228–1250.
  46. Obert J, Vercellino L, Van Der Gucht A, et al. (18) F-fluoro-deoxyglucose positron emission tomography-computed tomography in the management of adult multisystem Langerhans cell histiocytosis. Eur J Nucl Med Mol Imaging. 2017;44(4):598–610.
Labels
Haematology Internal medicine Clinical oncology
Editorial
Thrombotic microangiopathies
Castleman disease, one of the causes of chronic systemic inflammatory response as well as fluid retention, vasculitis and immune disorders sometimes – international criteria, 2017
The last days of patients with non-Hodgkin’s lymphoma - analysis of a sample of deceased patients over a period of 6 years (2012-2017) at one institution and the role of integrating palliative care
Storing red cells at non-standard temperature
Theralite and multiple myeloma – do we know the answer regarding treatment efficacy of a nephrologist with a haematologist?
Surprising findings in the bone marrow: what lies behind it?
Životní jubileum pana profesora Jiřího Mayera
Article was published in

Transfusion and Haematology Today

Issue 2
2020 Issue 2
Most read in this issue
Topics Journals
Login
Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.

Login

Don‘t have an account?  Create new account

#ADS_BOTTOM_SCRIPTS#