| Autor: |
Narula, G., Bhagwat, R., Arora, B., Banavali, S. D., Pai, S. K., Nair, C. N., Seth, T., Laskar, S., Muckaden, M. A., Kurkure, P. A., Parikh, P. M., Banavali, Sd, Pai, Sk, Nair, Cn, Muckaden, Ma, Kurkure, Pa, Parikh, Pm |
| Předmět: |
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| Zdroj: |
Indian Journal of Cancer; Jul-Sep2007, Vol. 44 Issue 3, p93-98, 6p, 2 Charts, 4 Graphs |
| Abstrakt: |
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. [ABSTRACT FROM AUTHOR] |
| Databáze: |
Complementary Index |
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