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Malignancy-associated hemophagocytic lymphohistiocytosis in adults: a retrospective population-based analysis from a single center
Hematology Center Karolinska, Karolinska University Hospital Huddinge, Stockholm, Sweden.
2011 (English)In: Leukemia and Lymphoma, ISSN 1042-8194, Vol. 52, no 4, 613-619 p.Article in journal (Refereed) Published
Abstract [en]

A retrospective, population-based study was conducted to evaluate the incidence, clinical features, and outcome of malignancy-associated hemophagocytic lymphohistiocytosis (M-HLH) in adults. Between January 1996 and December 2009, eight out of 887 (0.9%) patients diagnosed with hematological malignancies developed aggressive M-HLH in an area inhabited by approximately 160,000 people. Thus the estimated annual incidence of M-HLH in adulthood was 0.36/100,000 individuals/year. The clinical course of M-HLH was aggressive in all patients. Six patients were treated with a modified HLH-94 protocol; three achieved remission (durable in one case) while the others did not respond and died within an average of 2.4 months (range 1.5-3.5) after M-HLH diagnosis. Infection complicating the course of M-HLH occurred in four (50%) patients, all of whom developed fulminant M-HLH and died. Although the small study population limits the results, the long observation period strengthens its value.

Place, publisher, year, edition, pages
2011. Vol. 52, no 4, 613-619 p.
National Category
Clinical Medicine
URN: urn:nbn:se:uu:diva-233811DOI: 10.3109/10428194.2010.551153PubMedID: 21299462OAI: oai:DiVA.org:uu-233811DiVA: diva2:754362
Available from: 2014-10-10 Created: 2014-10-10 Last updated: 2014-10-14Bibliographically approved

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