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Clinical characteristics, therapy response, and outcome of 51 adult patients with hematological malignancy-associated hemophagocytic lymphohistiocytosis: a single institution experience
Umeå University, Faculty of Medicine, Department of Radiation Sciences.
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2018 (English)In: Leukemia and Lymphoma, ISSN 1042-8194, E-ISSN 1029-2403, Vol. 59, no 8, p. 1840-1850Article in journal (Refereed) Published
Abstract [en]

Hemophagocytic lymphohistiocytosis (HLH) is an underdiagnosed but life-threatening syndrome of hyperinflammation often occurring in adults with hematological malignancies (hM-HLH). The aim of the study was to describe clinical characteristics, therapy response, and outcome of adults with hM-HLH. The study included 51 adults with hM-HLH aged 23–84 years. Hyperferritinemia ≥500 µg/L was present in 96% of patients. The serum concentration of sIL-2Rα ≥ 2400 U/mL was revealed in 94% of patients. Twenty-three patients (45%) responded to therapy and achieved remission of HLH. The probability of overall survival (OS) at 6, 12, 24, and 60 months after HLH diagnosis were 42, 20, 15, and 15%, respectively. Patients with HLH during chemotherapy showed longer OS (median 124 days) than the patients who had HLH solely attributed to malignancy (median 65 days), but this difference was not statistically significant. Awareness of HLH in lymphoid and myeloid malignancies is crucial for improved survival.

Place, publisher, year, edition, pages
Taylor & Francis, 2018. Vol. 59, no 8, p. 1840-1850
Keywords [en]
Hemophagocytic lymphohistiocytosis, hemophagocytic syndrome, hyperferritinemia, cytokines, hematological malignancy, survival
National Category
Hematology
Identifiers
URN: urn:nbn:se:umu:diva-151198DOI: 10.1080/10428194.2017.1403018ISI: 000437352000009PubMedID: 29295642Scopus ID: 2-s2.0-85039858583OAI: oai:DiVA.org:umu-151198DiVA, id: diva2:1245482
Available from: 2018-09-05 Created: 2018-09-05 Last updated: 2018-09-05Bibliographically approved

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Lorenz, Fryderyk

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CiteExportLink to record
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