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Clinico-biologic profile of Langerhans cell histiocytosis: a single institutional study
1Department of Medical Oncology, Tata Memorial Hospital, Parel, Mumbai - 400 012, Maharashtra, India.
Insights
Langerhans cell histiocytosis (LCH) management in India shows outcomes similar to global data, with a notable increase in lymphoreticular involvement. Most patients respond well to therapy, but some high-risk cases need novel treatment strategies.
Area of Science:
- Pediatric Oncology
- Hematology
- Rare Diseases
Background:
- Langerhans cell histiocytosis (LCH) is a rare clonal disorder of Langerhans cells with varied presentations and outcomes.
- Limited Indian studies exist on LCH, necessitating local data on disease patterns and management.
Purpose of the Study:
- To document the clinical experience and outcomes of LCH management at a single Indian institution.
- To analyze the disease spectrum, treatment approaches, and survival rates in Indian LCH patients.
Main Methods:
- Retrospective observational study of 52 LCH patients treated between 1987 and 2002.
- Risk stratification based on current criteria, recording disease patterns, management, and outcomes.
- Statistical analysis included Student's t-test, proportion tests, and Kaplan-Meier survival estimates.
Main Results:
- Median age at presentation was 3 years; 48% had Group I disease.
- Commonly affected organs included skeleton, skin, and lymphoreticular system.
- Projected 10-year survival was 63%, with 17% of survivors experiencing long-term sequelae.
Conclusions:
- Indian LCH patients exhibit a profile similar to international data, with higher lymphoreticular involvement.
- Most patients achieve favorable outcomes with current therapies.
- A subset of Group I patients requires innovative therapeutic strategies to improve outcomes.
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 enrollment in clinical trials with innovative therapeutic strategies to improve outcome.