{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T13:13:54Z","timestamp":1790169234344,"version":"4.1.0"},"reference-count":0,"publisher":"National Library of Serbia","issue":"1","license":[{"start":{"date-parts":[[2016,1,1]],"date-time":"2016-01-01T00:00:00Z","timestamp":1451606400000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-sa\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["VSP","VOJNOSANIT PREGL","VOJNOSANITETSKI PREG"],"published-print":{"date-parts":[[2016]]},"abstract":"Introduction. Erdheim-Chester disease (ECD) is a rare, systemic form of\n non-Langerhans cell histiocytosis of the juvenile xantho-granuloma family\n with characteristic bilateral symmetrical long bone osteosclerosis,\n associated with xanthogranulomatous extras-keletal organ involvement. In ECD,\n central nervous system (CNS) and orbital lesions are frequent, and more than\n half of ECD patients carry the V600E mutation of the proto-oncogene BRAF. The\n synchronous or metachronous development of ECD and Langerhans cell\n histiocytosis (LCH) in the same patients is rare, and the possible connection\n between them is still obscure. Cladribine is a purine substrate analogue that\n is toxic to lymphocytes and monocytes with good hematoencephalic penetration.\n Case report. We presented a 23-year-old man successfully treated with\n cladribine due to BRAF V600E-mutation-negative ECD with bilateral orbital and\n CNS involvement. ECD developed metachronously, 6 years after chemotherapy for\n multisystem LCH with complete disease remission and remaining central\n diabetes insipidus. During ECD treatment, the patient received 5 single-agent\n chemotherapy courses of cladribine (5 mg\/m2 for 5 consecutive days every 4\n weeks), with a reduction in dose to 4 mg\/m2 in a fifth course, delayed due to\n severe neutropenia and thoracic dermatomal herpes zoster infection following\n the fourth course. Radiologic signs of systemic and CNS disease started to\n resolve 3 months after the end of chemotherapy, and CNS lesions completely\n resolved within 2 years after the treatment. After 12-year follow-up, there\n was no recurrence or appearance of new systemic or CNS xanthogranu-lomatous\n lesions or second malignancies. Conclusion. In accordance with our findings\n and recommendations provided by other authors, cladribine can be considered\n an effective alternative treatment for ECD, especially with CNS involvement\n and BRAF V600E-mutation-negative status, when interferon-? as the first-line\n therapy fails.<\/jats:p>","DOI":"10.2298\/vsp140915037p","type":"journal-article","created":{"date-parts":[[2015,6,10]],"date-time":"2015-06-10T13:19:03Z","timestamp":1433942343000},"page":"83-87","source":"Crossref","is-referenced-by-count":11,"title":["Successful treatment with cladribine of Erdheim-Chester disease with orbital and central nervous system involvement developing after treatment of langerhans cell histiocytosis"],"prefix":"10.2298","volume":"73","author":[{"given":"Predrag","family":"Peric","sequence":"first","affiliation":[{"name":"Military Medical Academy, Clinic for Neurosurgery, Belgrade + University of Defence, Faculty of Medicine of the Military Medical Academy, Belgrade"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Branislav","family":"Antic","sequence":"additional","affiliation":[{"name":"Military Medical Academy, Clinic for Neurosurgery, Belgrade + University of Defence, Faculty of Medicine of the Military Medical Academy, Belgrade"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Slavica","family":"Knezevic-Usaj","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, Department of Pathology, Novi Sad"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olga","family":"Radic-Tasic","sequence":"additional","affiliation":[{"name":"Military Medical Academy, Institute for Pathology and Forensic Medicine, Belgrade"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sanja","family":"Radovinovic-Tasic","sequence":"additional","affiliation":[{"name":"Military Medical Academy, Institute for Radiology, Belgrade"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jasenka","family":"Vasic-Vilic","sequence":"additional","affiliation":[{"name":"Military Medical Academy, Institute for Radiology, Belgrade"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leposava","family":"Sekulovic","sequence":"additional","affiliation":[{"name":"University of Defence, Faculty of Medicine of the Military Medical Academy, Belgrade + Military Medical Academy, Institute for Radiology, Belgrade"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivera","family":"Tarabar","sequence":"additional","affiliation":[{"name":"University of Defence, Faculty of Medicine of the Military Medical Academy, Belgrade + Military Medical Academy, Clinic for Hematology, Belgrade"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ljiljana","family":"Tukic","sequence":"additional","affiliation":[{"name":"University of Defence, Faculty of Medicine of the Military Medical Academy, Belgrade + Military Medical Academy, Clinic for Hematology, Belgrade"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9939-3726","authenticated-orcid":false,"given":"Stevo","family":"Jovandic","sequence":"additional","affiliation":[{"name":"Military Medical Academy, Institute for Medical Research, Belgrade"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5118-4933","authenticated-orcid":false,"given":"Zvonko","family":"Magic","sequence":"additional","affiliation":[{"name":"University of Defence, Faculty of Medicine of the Military Medical Academy, Belgrade + Military Medical Academy,Institute for Medical Research, Belgrade"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1078","container-title":["Vojnosanitetski pregled","Military Medical and Pharmaceutical Journal of Serbia"],"original-title":[],"language":"en","deposited":{"date-parts":[[2023,5,29]],"date-time":"2023-05-29T11:15:09Z","timestamp":1685358909000},"score":1,"resource":{"primary":{"URL":"https:\/\/doiserbia.nb.rs\/Article.aspx?ID=0042-84501500037P"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016]]},"references-count":0,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2016]]}},"URL":"https:\/\/doi.org\/10.2298\/vsp140915037p","relation":{},"ISSN":["0042-8450","2406-0720"],"issn-type":[{"value":"0042-8450","type":"print"},{"value":"2406-0720","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016]]}}}