Official Journal Health Science of Prince of Songkla University

  • Home
  • Search
  • Current
  • Archives
  • Announcements
  • Guide for Authors
  • Publication Ethics
  • Editorial Board
  • Submit
  • About
  • Contact
  • Online-first Articles
  • EVENTS
  • Review Process
Home > Vol 43, No 1 (2025) > Vathana

Clinical Outcomes of Langerhans Cell Histiocytosis: A Single-Center Experience

Nassawee Vathana, Siriporn Thitipolpun, Jassada Buaboonnam, Kleebsabai Sanpakit, Kamon Phuakpet

Abstract

Objective: To evaluate the clinical outcome of Langerhans cell histiocytosis (LCH) in countries with limited resources.
Material and Methods: A retrospective chart review of patients <15 years old, diagnosed with LCH and treated at Siriraj Hospital; from January 1, 2002 until December 31, 2016, was performed. The patients’ demographic data, treatment protocol, and efficacy of treatment were collected and analyzed.
Results: LCH was diagnosed in 38 patients, with a median age of 1.9 years old; of whom, 24 had multisystem disease (MS) and 14 had single system disease (SS). In the MS group, 16 patients had risk of organ (RO) involvement, with hepatic involvement being most common. The prevalence of central diabetes insipidus was 21.0%. Reactivation was observed in 11 patients (28.9%). RO involvement of the hematopoietic system (p-value=0.016), spleen (p-value=0.031), and MS (p-value=0.001) were significantly associated with a poor response to induction therapy. RO involvement of the hematopoietic system (p-value=0.016), liver (p-value=0.0), and spleen (p-value=0.005), as well as MS (p-value=0.013) were significantly associated with reactivation risk. The 5-year event-free survival rate of patients with and without RO involvement were 9.2% and 88.3%, respectively. The 5-year overall survival rate of patients with and without RO involvement were 82.5% and 96.4%, respectively.
Conclusion: The prevalence of MS and RO involvement seemed high in this cohort; however, the outcomes were comparable to other Asian studies. Novel treatment for RO involvement may improve clinical outcomes.

 Keywords

children; Langerhans cell histiocytosis; LCH, Thailand; treatment

 Full Text:

PDF

References

Emile JF, Abla O, Fraitag S, Horne A, Haroche J, Donadieu J, et al. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood 2016;127:2672-81.

Allen CE, Ladisch S, McClain KL. How I treat Langerhans cell histiocytosis. Blood 2015;126:26-35.

Galluzzo ML, Braier J, Rosenzweig SD, Garcia de Dávila MT, Rosso D. Bone marrow findings at diagnosis in patients with multisystem Langerhans cell histiocytosis. Pediatr Dev Pathol 2010;13:101-6.

Gadner H, Grois N, Potschger U, Minkov M, Arico M, Braier J, et al. Improved outcome in multisystem Langerhans cell histiocytosis is associated with therapy intensification. Blood 2008;111:2556-62.

Gadner H, Minkov M, Grois N, Potschger U, Thiem E, Arico M, et al. Therapy prolongation improves outcome in multisystem Langerhans cell histiocytosis. Blood 2013;121:5006-14.

Monsereenusorn C, Rodriguez-Galindo C. Clinical characteristics and treatment of Langerhans cell histiocytosis. Hematol Oncol Clin North Am 2015;29:853-73.

Morimoto A, Ikushima S, Kinugawa N, Ishii E, Kohdera U, Sako M, et al. Improved outcome in the treatment of pediatric multifocal langerhans cell histiocytosis: results from the japan langerhans cell histiocytosis study group-96 protocol study. Cancer 2006;107:613-9.

Takpradit C, Vathana N, Narkbunnam N, Sanpakit K, Buaboonnam J. Bisphosphonate therapy for refractory Langerhans cell histiocytosis: a case report. J Med Assoc Thai 2015;98:1145-9.

Yağcı B, Varan A, Çağlar M, Söylemezoğlu F, Sungur A, Orhan D, et al. Langerhans xell histiocytosis: retrospective analysis of 217 cases in a single center. Pediatr Hematol Oncol 2008;25:399-408.

Kim BE, Koh KN, Suh JK, Im HJ, Song JS, Lee JW, et al. Clinical features and treatment outcomes of Langerhans cell histiocytosis: a nationwide survey from Korea Histiocytosis Working Party. J Pediatr Hematol Oncol 2014;36:125-33.

Su M, Gao YJ, Pan C, Chen J, Tang JY. Outcome of children with Langerhans cell histiocytosis and single-system involvement: a retrospective study at a single center in Shanghai, China. Pediatr Hematol Oncol 2018;35:385-92.

Salotti JA, Nanduri V, Pearce MS, Parker L, Lynn R, Windebank KP. Incidence and clinical features of Langerhans cell histiocytosis in the UK and Ireland. Arch Dis Child 2009;94:376-80.

Donadieu J, Brugieres L, Thomas C, Herbelin C, Bertrand Y,

Schmitt C, et al. A multicentre retrospective survey of Langerhans’ cell histiocytosis: 348 cases observed between 1983 and 1993. The French Langerhans’ Cell Histiocytosis Study Group. Arch Dis Child 1996;75:17-24.

Gao YJ, Su M, Tang JY, Pan C, Chen J. Treatment outcome of children with multisystem Langerhans cell histiocytosis: the experience of a single children’s hospital in Shanghai, China. J Pediatr Hematol Oncol 2018;40:e9-e12.

Ince D, Demirag B, Ozek G, Erbay A, Ortac R, Oymak Y, et al. Pediatric Langerhans cell histiocytosis: single center experience over a 17-year period. Turk J Pediatr 2016;58:349-55.

Guyot-Goubin A, Donadieu J, Barkaoui M, Bellec S, Thomas C, Clavel J. Descriptive epidemiology of childhood Langerhans cell histiocytosis in France, 2000-2004. Pediatr Blood Cancer 2008;51:71-5.

Narula G, Pradhan ND, Arora B, Banavali SD. Treatment of Langerhans cell histiocytosis with a modified risk-adapted protocol-experience from a tertiary cancer institute in India. Pediatr Blood Cancer 2018;65:e27028.

Grois N, Flucher-Wolfram B, Heitger A, Mostbeck GH, Hofmann J, Gadner H. Diabetes insipidus in Langerhans cell histiocytosis: results from the DAL-HX 83 study. Med Pediatr Oncol 1995;24:248-56.

Modan-Moses D, Weintraub M, Meyerovitch J, Segal-Lieberman G, Bielorai B, Shimon I. Hypopituitarism in Langerhans cell histiocytosis: seven cases and literature review. J Endocrinol Invest 2001;24:612-7.

Heritier S, Emile JF, Barkaoui MA, Thomas C, Fraitag S, Boudjemaa S, et al. BRAF mutation correlates with high-risk Langerhans cell histiocytosis and increased resistance to first-line therapy. J Clin Oncol 2016;34:3023-30.

Morimoto A, Shioda Y, Imamura T, Kanegane 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 JJ, Ramalingam L, Zacharin MR. Bisphosphonate-associated osteonecrosis of the jaw: Does it occur in children? Clin Endocrinol 2008;68:863-7.

Morimoto A, Shioda Y, Imamura T, Kudo K, Kawaguchi H, Sakashita K, et al. Intensified and prolonged therapy comprising cytarabine, vincristine and prednisolone improves outcome in patients with multisystem langerhans cell histiocytosis: results of the japan langerhans cell histiocytosis study group-02 protocol study. Int J Hematol 2016;104:99-109.

Ma J, Laird JH, Chau KW, Chelius MR, Lok BH, Yahalom J. Langerhans cell histiocytosis in adults is associated with a high prevalence of hematologic and solid malignancies. Cancer Med 2019;8:58-66.

Adu-Poku K, Thomas DW, Khan MK, Holgate CS, Smith ME. Langerhans cell histiocytosis in sequential discordant lymphoma. J Clin Pathol 2005;58:104-6.

Dehkordi NR, Rajabi P, Naimi A, Heidarpour M. Langerhans cell histiocytosis following Hodgkin lymphoma: a case report from Iran. J Res Med Sci 2010;15:58-61.

Egeler RM, Neglia JP, Puccetti DM, Brennan CA, Nesbit ME. Association of Langerhans cell histiocytosis with malignant neoplasms. Cancer 1993;71:865-73.

Greaves WO, Bueso-Ramos C, Fayad L. Classical Hodgkin’s lymphoma associated with Langerhans cell histiocytosis: multiagent chemotherapy resulted in histologic resolution of both the classical Hodgkin’s lymphoma and Langerhans cell proliferation components. J Clin Oncol 2011;29:e76-8.

Park IS, Park IK, Kim EK, Kim S, Jeon SR, Huh JR, et al. Langerhans cell histiocytosis followed by Hodgkin’s lymphoma. Korean J Intern Med 2012;27:459-62.

Safaei A, Bagheri M, Shahryari J, Noori S, Esmailzade E. Langerhans cell histiocytosis followed by Hodgkin lymphoma: a case report. Iran J Med Sci 2015;40:282-6.

DOI: http://dx.doi.org/10.31584/jhsmr.20241072

Refbacks

  • There are currently no refbacks.
Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

SUBMIT A PAPER

JHSMR accepts online submission through

AHR-iCON 2024

Journal Metrics


2020
Acceptance rate: 52%
2021
Acceptance rate: 27.8%
2022 (March)
Acceptance rate: 15.6%
2023 (June)
Acceptance rate: 23.6%
2024 (June)
Acceptance rate: 19%


Submission to final decision
74 days

Acceptance to publication
40 days

0.6
2024CiteScore
 
 
31st percentile
Powered by Scopus



 

 

SCImago Journal & Country Rank

About The Authors

Nassawee Vathana
Division of Hematology and Oncology, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok 10700,
Thailand

Siriporn Thitipolpun
Division of Hematology and Oncology, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok 10700,
Thailand

Jassada Buaboonnam
Division of Hematology and Oncology, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok 10700,
Thailand

Kleebsabai Sanpakit
Division of Hematology and Oncology, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok 10700,
Thailand

Kamon Phuakpet
Division of Hematology and Oncology, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok 10700,
Thailand

Article Tools
Abstract
Print this article
Indexing metadata
How to cite item
Email this article (Login required)
Email the author (Login required)

Supported by

 

JHSMR now Indexed in



Scopus logo.svg






Image result for crossref





PSUMJ Homepage

Keywords COVID-19 SARS-CoV-2 Thailand Vietnam anxiety children computed tomography depression diabetes diabetes mellitus elderly knowledge mental health mortality prevalence quality of life reliability risk factors stroke treatment validity
Journal Content

Browse
  • By Issue
  • By Author
  • By Title
Font Size

Open Journal Systems