Early response to chemotherapy in children with Langerhans cell histiocytosis predicts long-term disease-free survival

Simple item page
dc.contributor.authorKrishnan, V.P.en_US
dc.contributor.authorKanvinde, Purvaen_US
dc.contributor.authorPatel, Swatien_US
dc.contributor.authorGolwala, Zainaben_US
dc.contributor.authorSwami, Archanaen_US
dc.contributor.authorHiwarkar, Prashanten_US
dc.contributor.authorMudaliar, Sangeetaen_US
dc.date.accessioned2023-08-09T06:04:28Z
dc.date.available2023-08-09T06:04:28Z
dc.date.issued2023-03
dc.description.abstractBackground: 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 outcomesen_US
dc.identifier.affiliationsDepartment of Pediatric Hemato-Oncology, Bai Jerbai Wadia Hospital for Children, Mumbai, Maharashtra, Indiaen_US
dc.identifier.citationKrishnan 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): 134-139en_US
dc.identifier.issn0019-509X
dc.identifier.placeIndiaen_US
dc.identifier.urihttps://imsear.searo.who.int/handle/123456789/221767
dc.languageenen_US
dc.publisherWolters Kluwer - Medknowen_US
dc.relation.issuenumber1en_US
dc.relation.volume60en_US
dc.source.urihttps://doi.org/10.4103/ijc.IJC_67_20en_US
dc.subjectEarly responseen_US
dc.subjectLangerhan cell histiocytosisen_US
dc.subjectoverall survivalen_US
dc.subjecttargeted therapyen_US
dc.titleEarly response to chemotherapy in children with Langerhans cell histiocytosis predicts long-term disease-free survivalen_US
dc.typeJournal Articleen_US
Files
Original bundle
Now showing 1 - 1 of 1
Thumbnail Image
Name:
IJC2022v60n1p134.pdf
Size:
1.52 MB
Format:
Adobe Portable Document Format
Download
Collections
Indian Journal of Cancer