{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T07:14:34Z","timestamp":1792394074212,"version":"4.4.0"},"reference-count":24,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2016,6,3]],"date-time":"2016-06-03T00:00:00Z","timestamp":1464912000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Hematological Oncology"],"published-print":{"date-parts":[[2017,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Adult onset hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome, which can develop as a complication of many disorders. Early diagnosis is essential in order to avoid a fatal outcome. To confirm the diagnosis of acquired HLH made in a single institution series of adult patients with HLH\u201004 criteria, we applied the HScore and evaluated prognostic factors associated with clinical outcome.<\/jats:p><jats:p>The median age of 35 patients was 54 (range 17\u201381), M\/F ratio was 20\/15. In 26\/35 (74.3%) patients, an underlying haematological disease was present (2 Multicentric Castleman Disease, 10 B\u2010cell Non\u2010Hodgkin Lymphoma [NHL] and 14 T\/NK\u2010cell NHL); an autoimmune disorder was observed in four (11.4%) patients (one Still Disease, one undifferentiated connective tissue disease and two haemolytic anaemia); in five (14.3%), no underlying disease was identified. A concomitant infection by EBV was observed in 10 patients (28.6%), CMV in 8 (22.9%), HHV8 in 6 (17.1%) and HIV in 1 (2.9%). Hyperferritinemia, fever and splenomegaly were present in more than 90% of patients, whereas bone marrow hemophagocytosis in 51% of cases only. According to HScore, 34\/35 patients had a &gt;75% and 32\/35 &gt;93% probability of HLH. Four\u2010year overall survival and HLH\u2010free survival were 17.8% (CI 1.9\u201333.8) and 23.8% (CI 7.3\u201340.3), respectively. By multivariate analysis, presence of oedema and hyperbilirubinemia were predictors of death, whereas there was a statistically significant trend for viral infection as predictor of poor prognosis. B\u2010NHL diagnosis was confirmed as associated to a better prognosis in comparison with T\/NK lymphoma (4\u2010year HFS 53.3% vs. 0%, p\u2009=\u20090.09) and similar to other aetiologies. Copyright \u00a9 2016 John Wiley &amp; Sons, Ltd.<\/jats:p>","DOI":"10.1002\/hon.2314","type":"journal-article","created":{"date-parts":[[2016,6,3]],"date-time":"2016-06-03T07:06:53Z","timestamp":1464937613000},"page":"828-834","source":"Crossref","is-referenced-by-count":41,"title":["Adult onset hemophagocytic lymphohistiocytosis prognosis is affected by underlying disease and coexisting viral infection: analysis of a single institution series of 35 patients"],"prefix":"10.1002","volume":"35","author":[{"given":"Chiara","family":"Cattaneo","sequence":"first","affiliation":[{"name":"Hematology Spedali Civili  Brescia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margherita","family":"Oberti","sequence":"additional","affiliation":[{"name":"Hematology Spedali Civili  Brescia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Skert","sequence":"additional","affiliation":[{"name":"Unit of Blood Diseases and Stem Cell 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