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However, we lack data on long\u2010term effects of 2CDA treatment, including the disease reactivation rate, permanent sequelae and long\u2010term tolerance. This study included 44 children from the French LCH registry, treated for a RO\u2212 LCH with 2CDA monotherapy (median number of six courses). The median age at the beginning of 2CDA was 3\u00b76\u00a0years (range, 0\u00b73\u201319\u00b77\u00a0years) and the median follow\u2010up after was 5\u00b74\u00a0years (range, 0\u00b76\u201315\u00b71\u00a0years). Objective response to 2CDA was observed in 25 patients (56\u00b78%), while six patients (13\u00b76%) had stable disease and 13 patients (29\u00b75%) exhibited progressive disease. Among patients without progression, only two experienced disease reactivation after 2CDA discontinuation. The five\u2010year cumulative incidence of disease progression or reactivation after 2CDA therapy initiation was 34\u00b73%. The lymphopenia reported in all cases [72% below absolute lymphocyte count (ALC) of 0\u00b75\u00a0G\/l], was addressed with appropriate prophylactic measures. Other toxicities above grade 2 were uncommon, and no second malignant neoplasm or neuropathy was reported. The five\u2010year overall survival was 97\u00b77%. In conclusion, we could confirm that 2CDA monotherapy was a beneficial long\u2010term therapy for treating patients with RO\u2212 LCH. Appropriate management of induced immune deficiency is mandatory.<\/jats:p>","DOI":"10.1111\/bjh.16944","type":"journal-article","created":{"date-parts":[[2020,7,23]],"date-time":"2020-07-23T01:29:57Z","timestamp":1595467797000},"page":"825-834","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["Long\u2010term follow\u2010up of children with risk organ\u2010negative Langerhans cell histiocytosis after 2\u2010chlorodeoxyadenosine treatment"],"prefix":"10.1111","volume":"191","author":[{"given":"Mohamed\u2010Aziz","family":"Barkaoui","sequence":"first","affiliation":[{"name":"French Reference Center for Langerhans Cell Histiocytosis Trousseau Hospital Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emma","family":"Queheille","sequence":"additional","affiliation":[{"name":"French Reference Center for Langerhans Cell Histiocytosis Trousseau Hospital Paris 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