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Annals of Hematology

, Volume 91, Issue 6, pp 897–904 | Cite as

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

  • Han-Seung Park
  • Dae-Young KimEmail author
  • 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
  • Young-Suk Lim
  • Seongsoo Jang
  • Chan-Jeoung Park
  • Hyun-Sook Chi
  • Kyoo-Hyung Lee
Original Article

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.

Keywords

Hemophagocytic syndrome Hemophagocytic lymphohistiocytosis Fibrinogen Prognosis 

Notes

Acknowledgments

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

Conflicts of interest

The authors declare no conflicts of interest.

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Copyright information

© Springer-Verlag 2011

Authors and Affiliations

  • Han-Seung Park
    • 1
  • Dae-Young Kim
    • 1
    Email author
  • Je-Hwan Lee
    • 1
  • Jung-Hee Lee
    • 1
  • Sung-Doo Kim
    • 1
  • Young-Hun Park
    • 1
  • Jae Seok Lee
    • 1
  • Bo Youn Kim
    • 1
  • Mijin Jeon
    • 1
  • Young-Ah Kang
    • 1
  • Young-Shin Lee
    • 1
  • Miee Seol
    • 1
  • Yeon-Joo Lee
    • 1
  • Young-Suk Lim
    • 2
  • Seongsoo Jang
    • 3
  • Chan-Jeoung Park
    • 3
  • Hyun-Sook Chi
    • 3
  • Kyoo-Hyung Lee
    • 1
  1. 1.Department of HematologyAsan Medical Center, University of Ulsan College of MedicineSeoulRepublic of Korea
  2. 2.Department of GastroenterologyAsan Medical Center, University of Ulsan College of MedicineSeoulRepublic of Korea
  3. 3.Department of Laboratory MedicineAsan Medical Center, University of Ulsan College of MedicineSeoulRepublic of Korea

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