Optimal therapy for adults with Langerhans cell histiocytosis bone lesions.

<h4>Background</h4>There is little data on treatment of Langerhans cell histiocytosis (LCH) in adults. Available data is on small numbers of patients with short follow-up times and no comparison of results from different treatment regimens. We analyzed the responses of adult LCH patients...

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Autores principales: Maria A Cantu, Philip J Lupo, Mrinalini Bilgi, M John Hicks, Carl E Allen, Kenneth L McClain
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Publicado: Public Library of Science (PLoS) 2012
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spelling oai:doaj.org-article:5fff2a5e14de401c839437d6d4b8e1b72021-11-18T07:08:37ZOptimal therapy for adults with Langerhans cell histiocytosis bone lesions.1932-620310.1371/journal.pone.0043257https://doaj.org/article/5fff2a5e14de401c839437d6d4b8e1b72012-01-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/pmid/22916233/pdf/?tool=EBIhttps://doaj.org/toc/1932-6203<h4>Background</h4>There is little data on treatment of Langerhans cell histiocytosis (LCH) in adults. Available data is on small numbers of patients with short follow-up times and no comparison of results from different treatment regimens. We analyzed the responses of adult LCH patients with bone lesions to three primary chemotherapy treatments to define the optimal one.<h4>Methods and findings</h4>Fifty-eight adult patients with bone lesions, either as a solitary site or as a component of multisystem disease, were analyzed for disease location and response to surgery, curettage, steroids, radiation, vinblastine/prednisone, 2-Chlorodeoxyadenosine (2-CdA), or cytosine arabinoside (ARA-C). The mean age of patients was 32 years, with equal gender distribution. Twenty-nine patients had 1 lesion; 16, 2 lesions; 5, 3 lesions; and 8 had 4 or more. Most bone lesions were in the skull, spine, or jaw. Chemotherapy, surgery, curettage, or radiation, but not steroids alone, achieved improvement or resolution of lesions in a majority of patients. Comparison of the three chemotherapy regimens revealed 84% of patients treated with vinblastine/prednisone either did not respond or relapsed within a year, whereas 59% of patients treated with 2-CdA and 21% treated with ARA-C failed. Toxicity was worse with the vinblastine/prednisone group as 75% had grade 3-4 neuropathy. Grade 3-4 cytopenias occurred in 37% of the 2-CdA -treated patients and 20% of the ARA-C-treated patients. The major limitation of this study is it is retrospective and not a clinical trial.<h4>Conclusions</h4>ARA-C is an effective and minimally toxic treatment for LCH bone lesions in adults. In contrast, vinblastine/prednisone results in poor overall responses and excessive toxicity.Maria A CantuPhilip J LupoMrinalini BilgiM John HicksCarl E AllenKenneth L McClainPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 7, Iss 8, p e43257 (2012)
institution DOAJ
collection DOAJ
language EN
topic Medicine
R
Science
Q
spellingShingle Medicine
R
Science
Q
Maria A Cantu
Philip J Lupo
Mrinalini Bilgi
M John Hicks
Carl E Allen
Kenneth L McClain
Optimal therapy for adults with Langerhans cell histiocytosis bone lesions.
description <h4>Background</h4>There is little data on treatment of Langerhans cell histiocytosis (LCH) in adults. Available data is on small numbers of patients with short follow-up times and no comparison of results from different treatment regimens. We analyzed the responses of adult LCH patients with bone lesions to three primary chemotherapy treatments to define the optimal one.<h4>Methods and findings</h4>Fifty-eight adult patients with bone lesions, either as a solitary site or as a component of multisystem disease, were analyzed for disease location and response to surgery, curettage, steroids, radiation, vinblastine/prednisone, 2-Chlorodeoxyadenosine (2-CdA), or cytosine arabinoside (ARA-C). The mean age of patients was 32 years, with equal gender distribution. Twenty-nine patients had 1 lesion; 16, 2 lesions; 5, 3 lesions; and 8 had 4 or more. Most bone lesions were in the skull, spine, or jaw. Chemotherapy, surgery, curettage, or radiation, but not steroids alone, achieved improvement or resolution of lesions in a majority of patients. Comparison of the three chemotherapy regimens revealed 84% of patients treated with vinblastine/prednisone either did not respond or relapsed within a year, whereas 59% of patients treated with 2-CdA and 21% treated with ARA-C failed. Toxicity was worse with the vinblastine/prednisone group as 75% had grade 3-4 neuropathy. Grade 3-4 cytopenias occurred in 37% of the 2-CdA -treated patients and 20% of the ARA-C-treated patients. The major limitation of this study is it is retrospective and not a clinical trial.<h4>Conclusions</h4>ARA-C is an effective and minimally toxic treatment for LCH bone lesions in adults. In contrast, vinblastine/prednisone results in poor overall responses and excessive toxicity.
format article
author Maria A Cantu
Philip J Lupo
Mrinalini Bilgi
M John Hicks
Carl E Allen
Kenneth L McClain
author_facet Maria A Cantu
Philip J Lupo
Mrinalini Bilgi
M John Hicks
Carl E Allen
Kenneth L McClain
author_sort Maria A Cantu
title Optimal therapy for adults with Langerhans cell histiocytosis bone lesions.
title_short Optimal therapy for adults with Langerhans cell histiocytosis bone lesions.
title_full Optimal therapy for adults with Langerhans cell histiocytosis bone lesions.
title_fullStr Optimal therapy for adults with Langerhans cell histiocytosis bone lesions.
title_full_unstemmed Optimal therapy for adults with Langerhans cell histiocytosis bone lesions.
title_sort optimal therapy for adults with langerhans cell histiocytosis bone lesions.
publisher Public Library of Science (PLoS)
publishDate 2012
url https://doaj.org/article/5fff2a5e14de401c839437d6d4b8e1b7
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