ArticleBMC cancer2015
High efficacy of the MACOP-B regimen in the treatment of adult Langerhans cell histiocytosis, a 20 year experience.
Article in BMC cancer, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 8 papers.
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Who cites it
8 citing papers in PubMed, 15 citations in OpenAlex.
- Phase 2 study of oral thalidomide-cyclophosphamide-dexamethasone for recurrent/refractory adult Langerhans cell histiocytosis.Leukemia · 2022Trial
- Trial
- Article
- Analysis of thyroid involvement in children and adult Langerhans cell histiocytosis: An underestimated endocrine manifestation.Frontiers in endocrinology · 2022Article
- Histiocytic disorders.Nature reviews. Disease primers · 2021Review
- Langerhans cell histiocytosis in adults: Advances in pathophysiology and treatment.Cancer science · 2018Review
- An unusual case of adult-onset multi-systemic Langerhans cell histiocytosis with perianal and incident thyroid involvement.Endocrinology, diabetes & metabolism case reports · 2017Article
- [Diabetes insipidus as the first manifestation of Langerhans cell histiocytosis: a case report and literatures review].Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi · 2016Article
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Authors and funding
10 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundAdult Langerhans cell histiocytosis (LCH) is an orphan disease. Chemotherapy is usually reserved to patients presenting with single system multifocal (SS-m) or multisystem (MS) disease but due to the lack of randomized studies no standard first line therapy has been defined yet. Pediatric regimens based on the vinblastine/prednisone backbone are not well tolerated in adults and probably less effective. We previously demonstrated high efficacy of the dose dense polichemotherapy regimen MACOP-B in 7 adult patients with SS-m or MS-LCH, in terms of high response rate and durable responses. Here we report an update of these data with the purpose of evaluating the long term efficacy of MACOP-B in adult LCH.
methodsClinical data of all adult LCH patients (n = 17) diagnosed and treated at our Institution during the past 20-year period were retrospectively reviewed.
resultsA total of 11 patients (6 with SS-m and 5 with MS-LCH) were treated with MACOP-B from 1995 to 2014. The overall response rate was confirmed to be 100 %, with a complete response of 73 % and a partial response rate of 27 %. Overall progression free survival was 64 %, and disease free survival after achievement of initial CR was 87 %. Overall survival rate was 82 % after 6.7 years of median follow-up.
conclusionsThese data confirm high activity of MACOP-B in adult LCH, indicating that a substantial fraction of patients achieve long lasting responses and can be cured with this therapeutic approach.
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