Early response to chemotherapy in children with Langerhans cell histiocytosis predicts long-term disease-free survival
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Date
2023-03
Journal Title
Journal ISSN
Volume Title
Publisher
Wolters Kluwer - Medknow
Abstract
Background: Langerhans cell histiocytosis (LCH) is a rare clonal malignancy of the monocyte-macrophage system. Patients with lesions in 搑isk organs� have significantly higher risk of mortality than patients with lesions limited to 搉on-risk� sites. The influence of early response to therapy on long-term survival in this heterogeneous multi-system disease was analyzed. Methods: During a 7-year period, we retrospectively analyzed the findings in 24 consecutive patients who required systemic chemotherapy for LCH [single system with multifocal bone involvement and multisystem involvement with or without risk organ (RO) involvement]. All patients were started on vinblastine and prednisolone. Progressive disease was treated with salvage protocols or targeted therapy. Positron emission tomography-computed tomography (PET-CT)/conventional CT based response assessment was performed at week 6 of chemotherapy, and if needed after week 12 of chemotherapy. Results: MFO bone, MS ROneg, and MS ROpos LCH was observed in 3, 4, and 17 patients, respectively. Age range of patients varied from 1 month�years (median = 18 months). The EFS and OS were 100% and 100% for MFO bone, 50% and 100%, respectively, for MS ROneg and 35% and 52%, respectively, for MS ROpos. OS was 93% and 100% for CR attained at 6 and 12 weeks respectively regardless of the risk status (P < 0.01). Conclusion: Rapid early response, that is, complete remission at 6 and 12 weeks was associated with significantly improved overall survival. In slow responders, early salvage with alternative regimens or targeted therapy may result in better outcomes
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Keywords
Early response, Langerhan cell histiocytosis, overall survival, targeted therapy
Citation
Krishnan V.P., Kanvinde Purva, Patel Swati, Golwala Zainab, Swami Archana, Hiwarkar Prashant, Mudaliar Sangeeta. Early response to chemotherapy in children with Langerhans cell histiocytosis predicts long-term disease-free survival. Indian Journal of Cancer . 2023 Mar; 60(1): 134-139