Retrospective analysis of clinical manifestations and treatment outcomes of patients diagnosed with langerhans cell histiocytosis from a tertiary cancer hospital in South India

Roshan Koshy Jacob, Shashidhar V. Karpurmath, Manjunath Nandennavar, Veerendra Angadi


Background: Langerhans cell histiocytosis (LCH) comprises a diverse group of disorders where pathologic Langerhans cells accumulate in a variety of organs. Aims and objectives of the study is to analyse the clinical manifestations and treatment outcomes of patients diagnosed with LCH in a tertiary cancer hospital in South India.

Methods: Retrospective analysis of the case records of patients presenting with histological proven case of LCH over a period of 7 years from 2011 to 2018, being treated at Vydehi Institute of Medical Sciences and Research Centre.

Results: 10 patients with biopsy proven LCH were included. The median age of diagnosis was 8 years (range 1 to 73 years) and 3 patients aged 18 years or older at the time of diagnosis. The male: female ratio was 3:2. Multisystem involvement was found in 4 patients (40%) and Single system Involvement in remaining 6 patients. Isolated bone lesions were found in 4 patients (40%), 1 patient had isolated Lymph node involvement; 1 patient had oral cavity lesion. None of the 4 patients with multisystem diseases had skin/mucosal involvement; 3 had bony involvement, 2 patients had lung involvement. One patients with multisystem disease expired while 5 patients were lost to follow-up. 4 out of the 10 patients are on regular follow-up and are in remission.

Conclusions: Despite limitation by the retrospective nature, this descriptive study was done to provide further disease information regarding Indian population. Data from this study clearly confirms the known fact that most of the patients with Single System LCH have a very good response rate. Patients with multisystem disease have the highest risk of disease related mortality and morbidity as one among the 4 patients with multisystem disease died just after initiating treatment.


Indian, Langerhans cell histiocytosis, Multisystem, Single system

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Allen C, Merad M, McClain K. Langerhans-Cell Histiocytosis. New Engl J Medi. 2018;379(9):856-68.

Shih Y, Lin W, Shih I. Langerhans Cell Histiocytosis in Taiwan: A Retrospective Case Series in a Medical Center. Dermatol Sinica. 2009;27:93-102.

Allen C, Ladisch S, McClain K. How I treat Langerhans cell histiocytosis. Blood. 2015;126(1):26-35.

Menghani H, Morrison C. Treatment and Survival Analysis for pediatric patients with Langerhans Cell Histiocytosis - A Single Institution Review. Pediatr Blood Cancer. 2016 June 1;63:S18-S18.

Demellawy D, Young J, Nanassy J, Chernetsova E, Nasr A. Langerhans cell histiocytosis: a comprehensive review. Pathology. 2015;47(4):294-301.

Eden P, Abeyasinghe WA, Mufees MB, Jayasooriya PR. A series of 13 new cases of langerhans cell histiocytosis of the oral cavity: a master of disguise. Age. 2017;6(15):16-25.

Mahendra K, ManUpdesh Singh S, Shano N. Bone marrow infiltration in Langerhan’s cell histiocytosis - An unusual but important determinant for staging and treatment. Int J Hematol- Oncol Stem Cell Res. 2015;9(4):193-7.

Tseng F, Kuo C. Langerhans cell histiocytosis: a case report. Endocr Abstr. 2014

Rao S, Hegde S, Gopinath Thilak P, Babu G, Ajila V, Shetty U, et al. Langerhans cell histiocytosis in a 5-year-old girl: A case report and review of literature. Ind J Medi Paediatr Oncol. 2019;40(5):176

Rao D, Trivedi M, Havale R, Shrutha S. A rare and unusual case report of Langerhans cell histiocytosis. J Oral Maxillof Pathol. 2017;21(1):140.