Case Study on Langerhans Cell Histiocytosis of Bone

Main Article Content

Mouna Djerbi
Mouna Snoussi
Hela Mnif
Faten Frikha
Sameh Marzouk
Tahya Sallemi Boudawara
Zeineb Mnif
Zouhir Bahloul

Abstract

Aims: To precise, the epidemiological, clinical, para-clinical, therapeutic and prognostic characteristics of skeletal involvement in Langerhans cell histiocytosis

Materials and Methods: A retrospective and descriptive study of patients with Langerhans cell histiocytosis admitted in Internal Medicine Departments of Hedi Chaker University Hospital of Sfax between 1996 and 2018. Cases of Langerhans cell histiocytois confirmed with histo- pathological examination were included.

Results: Four cases of LCH with bone involvement were noted. All patients enrolled were male and the mean age at diagnosis was 23.25 years. The bone lesions were unifocal in two cases and multifocal with multisystemic LCH in the others. The treatment consisted of curettage in two cases and two patients received systemic therapy with corticosteroids and vinblastine respectively. The outcome was favorable in two patients with eosinophilic ganuloma and systemic replases were noted with novel bone lesions in two patients presenting the systemic form of the disease.

Conclusion: LCH is a rare disease in children and young adult males. In the present series, bone was the most frequently involved site. The circumstances of discovery of bone localization were the pain swelling lesion in different sites. Biopsy is necessary to obtain diagnosis confirmation. The prognosis of this pathology depends largely on early diagnosis, other organs affected and the response to treatment. The new class of BRAF inhibitors may be a promising therapeutic option in LCH which needs to be assessed in prospective studies mainly in bone lesions.

Keywords:
Langerhans cell histiocytois, bone involvement, adult.

Article Details

How to Cite
Djerbi, M., Snoussi, M., Mnif, H., Frikha, F., Marzouk, S., Boudawara, T., Mnif, Z., & Bahloul, Z. (2019). Case Study on Langerhans Cell Histiocytosis of Bone. Asian Journal of Orthopaedic Research, 2(3), 1-10. Retrieved from http://journalajorr.com/index.php/AJORR/article/view/30099
Section
Case Study

References

Stephan Jl. Histiocytoses langerhansiennes et non langerhansiennes. Arch Pediatr. 2002;9:934–41.

Lieberman PH, Jones CR, Steiman RM, et al. Langerhans cell (éosiniphilic) granulomatosis. A clinicopathologic study encompassing 50 years. Am J Surg Pathol. 1996;20:519-52.

Arico M, Egeler RM. Clinical aspects of Langerhans cell histiocytosis. Hematol Oncol Clin North Am. 1998;2:247-58.

Badalian-Very G, Vergilio JA, Degar BA, MacConaill LE, Brandner B, Calicchio ML, et al. Recurrent BRAF mutations in langerhans cell histiocytosis. Blood 2010; 116:1919–23.

Ng-Cheng-Hin B, O’hanlon-Brown C, Alifrangis C, Waxman J. Langerhans cell histiocytosis: Old disease new treatment. Q J Med. 2011;104:89– 96.

Geismann F, Emile JF, Donadieu J, Andry P, Thomas C, Brousse N. Aspects cliniques et physiopathologiques de l’histiocytose Langerhansienne: Une prolifération clonale de cellules dendriti-ques de Langerhans. John Libbey Euro text. Revue Hématologie. 1997;3(1):33- 43.

Oehler E, Leogite J, Hellal Lk, Feuillet B, Evenat F, Ghawche F. Bonelesions. Rev Med Interne. 2014;35:554–5.

Lahiani D, Hammami BK, Maaloul I, Frikha M, Baklouti S, Jlidi R et al. Histiocytoselan-gerhansienne osseuse multifocale: révélation tardive chez une femme de 76 ans.Rev Med Interne. 2008;29:249–51.

Islinger RB, Kuklo TR, Owens BD, Horan PJ, Choma TJ, Murphey MD, et al. Langerhans’ cell histiocytosis in patients older than 21 years. ClinOrthop Relat Res. 2000;379:231–5.

Kilpatrick SE, Wenger DE, Gilchrist GS, Shives TC, Wollan PC, Unni KK. Langerhans’ cell histiocytosis (histiocytosis X) of bone. A clinicopathologic analysis of 263 pediatric and adult cases. Cancer. 1995;76:2471–84.

Suonita K, Jean Francois B, Elisa ABL, Gustavo S, Anne C, Nathaly B. Skeletal involvement in langerhans cell histio-cytosis. Insights Imaging. 2013;4(5):569–579.

Bollini G, Jouve Jl, Launay F, Viehweger E. Manifestations orthopédiques des histio-cytoses langerhansiennes. Arch Pediatr. 2008;15:526–8.

Okamoto K, Ito J, Furusawa T, Sakai K, Tokiguchi S. Imaging of calvarial eosinophil granuloma. Neuroradiology. 1999;41:723-8.

Matrane A, Guensi A, Kebbou M. Histiocytose langerhansienne osseuses multifocale: Intérêt de la scintigraphie osseuse planaire. Médecinenucléaire. 2012;36:730-735.

Dhanu GR, Malay VT, Raghavendra H, Shrutha SP. A rare and unusual case report of Langerhans cell histiocytosis. J Oral Maxillofac Pathol. 2017;21(1):140-144.

Floman Y, Baron E, Mosheiff R, Mirovsky Y, Robin GC, Ramu N. Eosinophilic granuloma of the spine. J Pediatr Orthop B. 1997;6:260-5.

Garg B, Sharma V, Eachampati KK, Malhortha R, Bhan S. An unusual presentation of eosinophilic granuloma in an adult: A case report. J Orthop Surg (Hong Kong). 2006;14:81-3.

Green NE, Robeston WW, Kilroy AW. Eosinophilic granuloma of the spine with associated neural deficit. J Bone Joint Surg Am. 1980;62:1198–202.

Zachary Christopher MD, Odion Binitie MD, Evita Henderson Jackson MD, Joseph Perno MD, Rikesh J. Makanji MD. Langerhans cell histiocytosis of bone in an adult: A case report. Radiology Case Reports. 2018;13:310–314.

Azouz EM, Saigal G, Rodrigeuz MM, Podda A. Langerhans’ cell histiocytosis: Pathology, imaging and treatment of skeletal involvement. Pediatr Radiol. 2005;35:103–15.

Hermans R, De Foer B, Smet MH, Leysen J, Feenstra L, Fossien E, et al. Eosinophile granuloma of the head and neck: CT and MRI features in three cases. Pediater Radiol. 1994;24:33-6.

Weston WJ, Goodson GM. Vertebraplana (Calve). J Bone Joint Surg Br. 1959;41: 477-85.

PeresleginIa, Ustinowa U. Radiotherapy of eosinophilic granuloma of bone. Int J Radiat Oncol Biol Phys. 1981;7:317-21.

Morimoto A, Shioda Y, Imamura T, Kangane H, Sato T, Kudo K, et al. Nationwide survey of bisphosphonate therapy for children with reactivated Langerhans cell histiocytosis in Japan. Pediatr Blood Cancer. 2011;56:110–5.

Brown RE. Bisphosphonates as antial-veolar macrophage therapy in pulmonary Langerhans cell histiocytosis? Med Pediatr Oncol. 2001;36:641–3.

D’Souza MJ, Oettinger CW, Shah A, Tipping PG, Huang XR, Milton GV. Macrophage depletion by albumin micro-encapsulated clodronate: attenuation of cytokine release in macrophage-dependent glomerulonephritis. Drug DevInd Pharm. 1999;25:591–6.

Montella L, Merola C, Merola G, Petillo L, Palmieri G. Zoledronic acid in treatment of bone lesions by Langerhans cell histiocytosis. J Bone Miner Metab. 2009; 27:110-113.
[PMID: 19018458]
DOI: 10.1007/s00774-008-0001-2

Abdellatif T, Gwneal L, Julien H, Antoine N, Stephane D, Achille A, Vinblastine chemotherapy in adult patients with langerhans cell histiocytosis: A multicenter retrospective study. Orphanet Journal of Rare Diseases. 2017;12:95.

Tin Wai C, Wing K L, Frankie Wai Tsoi C, Shekhar Medhukar K, Winnie Chiu Wing C, Vincent Lee, et al. Late outcomes in children with Langerhans cell histiocytosis. Arch Dis Child. 2017;102(9):830-835.

Bollini G, Jouve Jl, Gentet JC, Jaquemier M, Bouyala JM. Bone lesions in histio-cytosis X. J Pediatr Orthop. 1994;11:469-77.

Diamond EL, Subbiah V, Lockhart AC, et al. Histiocytosis: Analysis of data from the histology- independent, phase 2, open- label VE- BASKET Study. JAMA Oncol. 2018;4:384-388.

Bhatia A, Ulaner G, Rampal R, et al. Single-agent daBRAFenib for BRAFV600 E-mutated histiocytosis. Haematologica. 2018;103:e177-e180.

Papapanagiotou M, Griewank KG, Hillen U, et al. Trametinib- induced remission of an MEK1- mutated langerhans cell histiocytosis. JCO Precis Oncol. 2017;1:1-5.

Masayuki kobayashi, Arinobo Tojo. Langerhans cell histiocytosis in adults. advances in physiopathology and treatment. Cancer Science. 2018;109: 3707–3713.

Carl CEA, Ladish SMC, Clain Kl. How I treat langerhans cell histiocytosis. Blood. 2015;126(1):26–35.

Carl E. Allen, Miriam Merad, Kenneth L. Langerhans-cell histiocytosis. N Engl J Med. 2018;379:856-868.

Michaloglou C, Vredeveld LC, Mooi WJ, Peeper DS. BRAF(E600) in benign and malignant human tumours. Oncogene. 2008;27:877-95

Cagnol S, Chambard JC. ERK and cell death: Mechanisms of ERK-induced cell death — apoptosis, autophagy and senescence. FEBS J. 2010;277:2-21.

Poulikakos PI, Zhang C, Bollag G, et al. RAF inhibitors transactivate RAF dimers and ERK signalling in cells with wild-type BRAF. Nature. 2010;464:427-30.

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;121:1495-500.

Hyman DM, Puzanov I, Subbiah V, et al. Vemurafenib in multiple nonmelanoma cancers with BRAF V600 mutations. N Engl J Med. 2015;373:726-36.