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Clinical features of adult patients with secondary hemophagocytic lymphohistiocytosis from causes other than lymphoma: an analysis of treatment outcome and prognostic factors

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Abstract

Although hemophagocytic syndrome (HS) featuring secondary hemophagocytic lymphohistiocytosis (HLH) has a grave prognosis, little is known about the natural course of the disease. Patients who showed the clinical features of HLH as well as tissue-proven hemophagocytosis when seen at Asan Medical Center between 1999 and 2010 were included in this analysis. Patients with proven lymphoma were excluded. The median age of our 23 study patients was 49 years. Epstein–Barr virus was suspected to have caused HS in 16 (70%) patients and hepatitis A virus in one patient. Twenty-two patients were treated, 13 according to the HLH protocol and nine using immunosuppressive agents such as corticosteroid and/or cyclosporine. Five patients undertook allogeneic hematopoietic cell transplantation (HCT) during their treatment-dependent relapse (n = 4) or responsive status (n = 1). After the median follow-up of 180 days, 17 (74%) died and six (26%) were alive. The median time from initial presentation until death was 41 days among those patients who died. The serum fibrinogen level ≥166 mg/dL determined at the initial visit was significantly associated with the survival time according to univariate analysis. The low histiocyte proportion in bone marrow and early initiation of treatment tended to correlate with a favorable outcome. On multivariate analysis, serum fibrinogen ≥166 mg/dL (hazard ratio, 0.175, P = 0.018) was an independent clinical factor for determining the patient survival time. Despite appropriate patient management, the outcome of HS featuring HLH was grave. The serum fibrinogen level at the initial presentation was significant, and selected patients obtained some benefit from allogeneic HCT.

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Acknowledgments

This study was supported by a grant (KG2010-10) Basic Research in Medicine, Korean Institute of Medicine & GlaxoSmithKline, Seoul, Korea.

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The authors declare no conflicts of interest.

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Authors and Affiliations

  1. Department of Hematology, Asan Medical Center, University of Ulsan College of Medicine, Asanbyeongwon-gil 86, Songpa-gu, Seoul, 138-736, Republic of Korea

    Han-Seung Park, Dae-Young Kim, Je-Hwan Lee, Jung-Hee Lee, Sung-Doo Kim, Young-Hun Park, Jae Seok Lee, Bo Youn Kim, Mijin Jeon, Young-Ah Kang, Young-Shin Lee, Miee Seol, Yeon-Joo Lee & Kyoo-Hyung Lee

  2. Department of Gastroenterology, Asan Medical Center, University of Ulsan College of Medicine, Asanbyeongwon-gil 86, Songpa-gu, Seoul, 138-736, Republic of Korea

    Young-Suk Lim

  3. Department of Laboratory Medicine, Asan Medical Center, University of Ulsan College of Medicine, Asanbyeongwon-gil 86, Songpa-gu, Seoul, 138-736, Republic of Korea

    Seongsoo Jang, Chan-Jeoung Park & Hyun-Sook Chi

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  1. Han-Seung Park
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Correspondence to Dae-Young Kim.

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Park, HS., Kim, DY., Lee, JH. et al. Clinical features of adult patients with secondary hemophagocytic lymphohistiocytosis from causes other than lymphoma: an analysis of treatment outcome and prognostic factors. Ann Hematol 91, 897–904 (2012). https://doi.org/10.1007/s00277-011-1380-3

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  • DOI: https://doi.org/10.1007/s00277-011-1380-3

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