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Annals of Hematology

, 86:493 | Cite as

Lymphoma-associated hemophagocytic syndrome: clinical features and treatment outcome

  • A-Reum Han
  • Hye Ran Lee
  • Byeong-Bae Park
  • In Gyu Hwang
  • Sarah Park
  • Sang Cheol Lee
  • Kihyun Kim
  • Ho Yeong Lim
  • Young H. Ko
  • Sun Hee Kim
  • Won Seog KimEmail author
Original Article

Abstract

The clinical features and prognostic factor of lymphoma-associated hemophagocytic syndrome (LAHS), diagnosed according to World Health Organization classification, were investigated by reviewing the clinical records of 29 patients between September 1994 and September 2006. Compared with patients with T or natural killer (NK)/T cell LAHS, patients with B cell LAHS were older (p = 0.022), were less likely to exhibit disseminated intravascular coagulation (DIC; p = 0.011), and had less direct involvement of bone marrow (p = 0.03). Clinical response was achieved in 15 (65.2%) and complete remission (CR) was achieved in 4 (17%) of 23 patients who received chemotherapy. Four patients received high-dose chemotherapy and autologous stem cell transplantation (A-SCT), and three of these four patients showed CR. The median survival was 36 days (95%CI, 20.2–51.8). Univariate analysis showed that poor performance status (p = 0.028), T or NK/T cell lymphoma (p = 0.016), presence of jaundice (p = 0.063), the presence of DIC (p = 0.002), and poor clinical response to treatment (p < 0.001) predicted poor overall survival. These data suggest that the clinical features differ significantly between B cell LAHS and T or NK/T cell LAHS. Intensive treatment including high-dose chemotherapy and A-SCT should be investigated.

Keywords

Hemophagocytic syndrome Lymphoma-associated hemophagocytic syndrome 

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Copyright information

© Springer-Verlag 2007

Authors and Affiliations

  • A-Reum Han
    • 1
  • Hye Ran Lee
    • 2
  • Byeong-Bae Park
    • 3
  • In Gyu Hwang
    • 3
  • Sarah Park
    • 3
  • Sang Cheol Lee
    • 3
  • Kihyun Kim
    • 3
  • Ho Yeong Lim
    • 3
  • Young H. Ko
    • 4
  • Sun Hee Kim
    • 5
  • Won Seog Kim
    • 3
    Email author
  1. 1.Department of Medicine, Samsung Medical CenterSungkyunkwan University School of MedicineSeoulSouth Korea
  2. 2.Division of Hematology–Oncology, Department of Medicine, Paik HospitalInje University School of MedicineSeoulSouth Korea
  3. 3.Division of Hematology–Oncology, Department of Medicine, Samsung Medical CenterSungkyunkwan University School of MedicineSeoulSouth Korea
  4. 4.Department of Pathology, Samsung Medical CenterSungkyunkwan University School of MedicineSeoulSouth Korea
  5. 5.Department of Laboratory Medicine, Samsung Medical CenterSungkyunkwan University School of MedicineSeoulSouth Korea

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