Early response to chemotherapy in children with Langerhans cell histiocytosis predicts long-term disease-free survival
dc.contributor.author | Krishnan, V.P. | en_US |
dc.contributor.author | Kanvinde, Purva | en_US |
dc.contributor.author | Patel, Swati | en_US |
dc.contributor.author | Golwala, Zainab | en_US |
dc.contributor.author | Swami, Archana | en_US |
dc.contributor.author | Hiwarkar, Prashant | en_US |
dc.contributor.author | Mudaliar, Sangeeta | en_US |
dc.date.accessioned | 2023-08-09T06:04:28Z | |
dc.date.available | 2023-08-09T06:04:28Z | |
dc.date.issued | 2023-03 | |
dc.description.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 | en_US |
dc.identifier.affiliations | Department of Pediatric Hemato-Oncology, Bai Jerbai Wadia Hospital for Children, Mumbai, Maharashtra, India | en_US |
dc.identifier.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 | en_US |
dc.identifier.issn | 0019-509X | |
dc.identifier.place | India | en_US |
dc.identifier.uri | https://imsear.searo.who.int/handle/123456789/221767 | |
dc.language | en | en_US |
dc.publisher | Wolters Kluwer - Medknow | en_US |
dc.relation.issuenumber | 1 | en_US |
dc.relation.volume | 60 | en_US |
dc.source.uri | https://doi.org/10.4103/ijc.IJC_67_20 | en_US |
dc.subject | Early response | en_US |
dc.subject | Langerhan cell histiocytosis | en_US |
dc.subject | overall survival | en_US |
dc.subject | targeted therapy | en_US |
dc.title | Early response to chemotherapy in children with Langerhans cell histiocytosis predicts long-term disease-free survival | en_US |
dc.type | Journal Article | en_US |
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