{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,20]],"date-time":"2026-05-20T00:12:53Z","timestamp":1779235973945,"version":"3.51.4"},"reference-count":0,"publisher":"SMW Supporting Association","issue":"2526","license":[{"start":{"date-parts":[[2015,6,14]],"date-time":"2015-06-14T00:00:00Z","timestamp":1434240000000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-sa\/4.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Swiss Med Wkly"],"abstract":"<jats:p>BACKGROUND: We retrospectively analysed charts of patients with blood ferritin level &gt;5000 \u00b5g\/l. The aim of the study was to look for the likelihood of haemophagocytic lymphohistiocytosis (HLH) in these patients.\n        METHODS: Forty-two patients demonstrated hyperferritinaemia and could be evaluated. The diagnosis of HLH was based on a recently published HScore and an earlier diagnostic algorithm.\n        RESULTS: According to the algorithm, 20 patients fulfilled the criteria for a diagnosis of HLH. However, patients with Still\u2019s disease have macrophage activation and, in this context, a rise in ferritin without having HLH. Fourteen patients with carcinoma, haematological malignancies or infection and hyperferritinaemia remained. Signs and symptoms were: systemic inflammatory response syndrome (SIRS 100%), fever (95%), cytopenia of \u22652 lines (70%), immunosuppression (61.5%), splenomegaly (50%), elevated liver enzymes (45%), lymphadenopathy (35%), hepatomegaly (30%). These are nonspecific parameters. Therefore HLH may be overdiagnosed. Using the HScore, only 10 patients had &gt;80% probability of having HLH. Patients demonstrating cytopenia of \u22652 cell lines had a &gt;60% mortality rate. Time to death was 13.8 days; death was most often due to multiorgan failure.\n        CONCLUSION: HScore reflects a higher specificity than the algorithm for diagnosing HLH. The discrepancy may indicate the difficulty that a specific marker still is missing. Hyperferritinaemia was strongly associated with HLH in patients with haematological or oncological malignancies. HLH may be underdiagnosed because the majority of these patients suffer from a severe underlying disease, which easily might suggest a flare or infection. In this population, hyperferritinaemia and SIRS should rise suspicion because mortality in HLH is high.<\/jats:p>","DOI":"10.4414\/smw.2015.14152","type":"journal-article","created":{"date-parts":[[2016,1,29]],"date-time":"2016-01-29T10:03:57Z","timestamp":1454061837000},"page":"w14152","source":"Crossref","is-referenced-by-count":6,"title":["Patients with an extraordinarily elevated serum ferritin: think of haemophagocytic lymphohistiocytosis"],"prefix":"10.57187","volume":"145","author":[{"given":"Marc","family":"Schweizer","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeroen  S.","family":"Goede","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Verena","family":"Briner","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"36756","published-online":{"date-parts":[[2015,6,14]]},"container-title":["Swiss Medical Weekly"],"original-title":[],"link":[{"URL":"https:\/\/smw.ch\/index.php\/smw\/article\/download\/2039\/2956","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/smw.ch\/index.php\/smw\/article\/download\/2039\/2957","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/smw.ch\/index.php\/smw\/article\/download\/2039\/2956","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,15]],"date-time":"2025-08-15T06:27:50Z","timestamp":1755239270000},"score":1,"resource":{"primary":{"URL":"https:\/\/smw.ch\/index.php\/smw\/article\/view\/2039"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,6,14]]},"references-count":0,"journal-issue":{"issue":"2526","published-online":{"date-parts":[[2015,6,15]]}},"URL":"https:\/\/doi.org\/10.4414\/smw.2015.14152","relation":{},"ISSN":["1424-3997"],"issn-type":[{"value":"1424-3997","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,6,14]]}}}