Intensified and prolonged therapy comprising cytarabine, vincristine and prednisolone improves outcome in patients with multisystem Langerhans cell histiocytosis: results of ... View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2016-07

AUTHORS

Akira Morimoto, Yoko Shioda, Toshihiko Imamura, Kazuko Kudo, Hiroshi Kawaguchi, Kazuo Sakashita, Masahiro Yasui, Yuhki Koga, Ryoji Kobayashi, Eiichi Ishii, Junichiro Fujimoto, Keizo Horibe, Fumio Bessho, Yukiko Tsunematsu, Shinsaku Imashuku

ABSTRACT

The JLSG-96 study reported very low mortality rates for children newly diagnosed with multifocal Langerhans cell histiocytosis (LCH). The JLSG-02 study was performed to further improve the prognosis from 2002 to 2009. The present study compared the therapeutic results of these two studies in terms of multisystem disease. All patients were treated with 6 weeks of the Induction A regimen, comprising cytarabine, vincristine and prednisolone, followed by maintenance therapy. Poor responders to Induction A were switched to Induction B. JLSG-02 has been revised from JLSG-96 in the following respects: prednisolone dosage during Induction A increased; duration of maintenance therapy extended from 24 to 48 weeks; cyclosporine introduced to Induction B for progressive disease. One hundred forty-seven children with multisystem LCH were evaluated. Of these, 84 were positive for risk of organ involvement (RO) and 63 were RO-negative. At the 6-week point, 76.2 % of RO+ and 93.7 % of RO- patients responded to Induction A. Five-year event-free survival (EFS) was 46.2 % [95 % confidence (CI), 35.5-56.9] for RO+ and 69.7 % (58.4-81.1) for RO-, which was significantly superior to that in JLSG-96 [26.8 % (13.3-40.4) and 38.9 % (16.4-61.4), respectively]. The intensified induction and prolonged maintenance regimens in JLSG-02 improved EFS in patients with multisystem LCH. More... »

PAGES

99-109

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/s12185-016-1993-3

DOI

http://dx.doi.org/10.1007/s12185-016-1993-3

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1020802816

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/27040279


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RDF/XML is a standard XML format for linked data.

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315 https://www.grid.ac/institutes/grid.410840.9 schema:alternateName Nagoya Medical Center
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331 schema:name National Center for Child Health and Development, Tokyo, Japan
332 Pediatric Cancer Center, National Center for Child Health and Development, Tokyo, Japan
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