Milen Minkov, Ulrike Pötschger, Nirav Thacker, Itziar Astigarraga, Jorge Braier, Jean Donadieu, Jan-Inge Henter, Thomas Lehrnbecher, Carlos Rodriguez-Galindo, Elena Sieni, Vasanta Nanduri, Cor van den Bos, Oussama Abla
- Objective: To evaluate the prognostic impact of gastrointestinal involvement on the survival of children with Langerhans cell histiocytosis (GI-LCH) registered with the international clinical trials of the Histiocyte Society. Study design: This was a retrospective analysis of 2414 pediatric patients registered onto the consecutive trials DAL-HX 83, DAL-HX 90, LCH-I, LCH-II, and LCH-III. Results: Among the 1289 patients with single-system LCH, there was no single case confined to the GI tract; 114 of 1125 (10%) patients with multisystem LCH (MS-LCH) had GI-LCH at initial presentation. GI-LCH was significantly more common in children aged <2 years at diagnosis (13% vs 6% in those aged >2 years; P < .001) and in those with risk organ involvement (15% vs 6% in those without risk organ involvement; P < .001). The 5-year overall survival (OS) in patients without risk organ involvement was excellent irrespective of GI disease (98% vs 97% in patients with GI-LCH; P = .789). In patients with risk organ involvement, the 5-year OS was 51% in 70 patients with GI-LCH vs 72% in 394 patients without GI-LCH (P < .001). Conclusions: GI-LCH has an additive unfavorable prognostic impact in children with MS-LCH and risk organ involvement. The emerding need for more intensive or alternative treatments mandates prospective evaluation.
MetadatenAuthor: | Milen Minkov, Ulrike Pötschger, Nirav Thacker, Itziar Astigarraga, Jorge Braier, Jean Donadieu, Jan-Inge Henter, Thomas LehrnbecherORCiDGND, Carlos Rodriguez-Galindo, Elena Sieni, Vasanta Nanduri, Cor van den Bos, Oussama Abla |
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URN: | urn:nbn:de:hebis:30:3-645376 |
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DOI: | https://doi.org/10.1016/j.jpeds.2021.06.016 |
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ISSN: | 1097-6833 |
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Pubmed Id: | https://pubmed.ncbi.nlm.nih.gov/34146548 |
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Parent Title (English): | The journal of pediatrics |
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Publisher: | Elsevier |
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Place of publication: | Frankfurt a. M. [u.a.] |
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Document Type: | Article |
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Language: | English |
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Date of Publication (online): | 2021/06/16 |
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Date of first Publication: | 2021/06/16 |
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Publishing Institution: | Universitätsbibliothek Johann Christian Senckenberg |
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Contributing Corporation: | LCH Study Group of the Histiocyte Society |
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Release Date: | 2022/01/24 |
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Tag: | Langerhans cell histiocytosis; children; digestive tract; gastrointestinal tract; prognosis |
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Volume: | 237 |
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Page Number: | 9 |
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First Page: | 65 |
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Last Page: | 70.e.3 |
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Note: | Financial support was provided for the management of the original clinical trials of the Histiocyte Society by the Histiocytosis Association and the Children's Cancer Research Institute (Vienna, Austria), both nonprofit organizations. |
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HeBIS-PPN: | 491338929 |
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Institutes: | Medizin / Medizin |
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Dewey Decimal Classification: | 6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 610 Medizin und Gesundheit |
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Sammlungen: | Universitätspublikationen |
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Licence (German): | Creative Commons - Namensnennung 4.0 |
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