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 ORIGINAL ARTICLE
Year : 2007  |  Volume : 44  |  Issue : 3  |  Page : 93-98

Clinico-biologic profile of Langerhans cell histiocytosis: A single institutional study


1 Department of Pediatrics, INHS Asvini, Colaba, India
2 Department of Medical Oncology, Tata Memorial Hospital, Parel, Mumbai - 400 012, Maharashtra, India
3 Department of Pathology, Tata Memorial Hospital, Parel, Mumbai - 400 012, Maharashtra, India
4 Department of Medical Oncology and Radiation Oncology, Tata Memorial Hospital, Parel, Mumbai - 400 012, Maharashtra, India
5 Department of Radiation Oncology, Tata Memorial Hospital, Parel, Mumbai - 400 012, Maharashtra, India

Correspondence Address:
P A Kurkure
Department of Medical Oncology, Tata Memorial Hospital, Parel, Mumbai - 400 012, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-509X.38939

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Context: Langerhans cell histiocytosis (LCH) is a rare atypical cellular disorder characterized by clonal proliferation of Langerhans cells leading to myriad clinical presentations and highly variable outcomes. There is a paucity of Indian studies on this subject. Aim: To present the experience of management of LCH at a single institution. Settings and Design: This is a retrospective observational study of patients with LCH who presented at the Tata Memorial Hospital between January 1987 and December 2002. Materials and Methods: Fifty-two patients with LCH were treated in the study period. Due to the long observation period and variability in diagnostic and therapeutic protocols, the patients were risk-stratified based on present criteria. The disease pattern, management approaches and treatment outcomes of patients were recorded. Statistical Analysis Used: Statistical analyses were done using Student's 't' test, test for proportion and survival estimates based on the Kaplan-Meier method. Results: The median age at presentation was 3 years and more than 48% of the patients had Group I disease. Skeleton, skin and lymphoreticular system were the commonly involved organs. Majority (80%) required some form of therapy. The projected overall survival is 63% at 10 years and mean survival is 118 months. Seventeen percent of surviving patients developed long-term sequelae. Conclusions: The clinico-biologic profile of LCH patients in India is largely similar to international patterns except a higher incidence of lymphoreticular involvement. Majority of the patients respond favorably to therapy and have a good outcome, except a subset of Group I patients who warrant enrolment in clinical trials with innovative therapeutic strategies to improve outcome.






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