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Secondary haemophagocytic lymphohistiocytosis: Experience from the Uppsala University Hospital
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
2015 (English)In: Upsala Journal of Medical Sciences, ISSN 0300-9734, E-ISSN 2000-1967, Vol. 120, no 4, 257-262 p.Article in journal (Refereed) Published
Abstract [en]

BACKGROUND: Haemophagocytic lymphohistiocytosis (HLH) is a rare clinical syndrome characterized by fever, hepatosplenomegaly, cytopenia, and progressive multiple-organ failure. HLH in adults is often secondary to autoimmune diseases, cancer, or infections in contrast to familial HLH. Treatment of secondary HLH is directed against the triggering disease in addition to immunosuppressive therapy, the latter commonly according to the HLH-2004 protocol.

METHODS: We conducted a retrospective study to identify triggering diseases, disease-specific and immunosuppressive therapy administered, and prognosis in adult patients with secondary HLH. Patient data were collected from October 2010 to January 2015.

RESULTS: Ten adult patients with secondary HLH were identified. Seven were men, and the median age at diagnosis was 62 years. Five cases were triggered by malignant disease and five by infection. The median patient fulfilled five of the eight HLH-2004 diagnostic criteria. All patients fulfilled the criteria fever, cytopenia, and ferritin >500 µg/L. Median time from hospital admission to HLH diagnosis was 20 days. Four patients received immunosuppressive therapy according to the HLH-2004 protocol. The prognosis was dismal, especially for the patients with malignancy-associated HLH, of whom all died.

CONCLUSION: HLH should be suspected in patients who present with fever, cytopenia, and ferritin >500 µg/L. Secondary HLH has a dismal prognosis. None of the patients with HLH triggered by malignancy survived. Achieving remission of the triggering disease seems to be important for a favourable outcome as, in all surviving patients, the haemophagocytic syndrome resolved after remission of the underlying infection.

Place, publisher, year, edition, pages
2015. Vol. 120, no 4, 257-262 p.
Keyword [en]
Haemophagocytic lymphohistiocytosis; HLH-2004 protocol
National Category
Medical and Health Sciences Hematology
URN: urn:nbn:se:uu:diva-266755DOI: 10.3109/03009734.2015.1064500ISI: 000365684900005PubMedID: 26212358OAI: oai:DiVA.org:uu-266755DiVA: diva2:868407
Available from: 2015-11-10 Created: 2015-11-10 Last updated: 2016-01-11Bibliographically approved

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