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Clinical and laboratory features are characteristic, but unspecific; thus awareness of FHL is important for early diagnosis. FHL is rapidly fatal without treatment. Standard\u2010of\u2010care therapy is etoposide and corticosteroids, followed by haematopoietic stem cell transplantation (HSCT).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>FHL has become a curable disease with present treatment. Additional cytokine\u2010directed therapy still has to prove its value. Earlier HSCT and less toxic conditioning regimens will lead to improved cure rates.<\/jats:p><\/jats:sec>","DOI":"10.1111\/apa.15889","type":"journal-article","created":{"date-parts":[[2021,4,28]],"date-time":"2021-04-28T07:45:04Z","timestamp":1619595904000},"page":"2723-2728","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Clinical features, diagnosis and therapy of familial haemophagocytic lymphohistiocytosis"],"prefix":"10.1111","volume":"110","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5397-3047","authenticated-orcid":false,"given":"Gritta E.","family":"Janka","sequence":"first","affiliation":[{"name":"Department of Paediatric Haematology and Oncology University Medical Center Hamburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maurizio","family":"Aric\u00f2","sequence":"additional","affiliation":[{"name":"Strategic Direction Staff, Children's Hospital Giovanni XXIII, Azienda Ospedaliero Universitaria Consorziale Policlinico Bari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,5,19]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.27.136.519"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.coi.2017.08.004"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00443367"},{"key":"e_1_2_11_5_1","first-page":"197","article-title":"Hemophagocytic lymphohistiocytosis. 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