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Lymphoma-associated hemophagocytic syndrome: clinical features and treatment outcome

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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.

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Authors and Affiliations

  1. Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea

    A-Reum Han

  2. Division of Hematology–Oncology, Department of Medicine, Paik Hospital, Inje University School of Medicine, Seoul, South Korea

    Hye Ran Lee

  3. Division of Hematology–Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Ilwon-Dong, Kangnam-Ku, Seoul, 135-710, South Korea

    Byeong-Bae Park, In Gyu Hwang, Sarah Park, Sang Cheol Lee, Kihyun Kim, Ho Yeong Lim & Won Seog Kim

  4. Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea

    Young H. Ko

  5. Department of Laboratory Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea

    Sun Hee Kim

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  1. A-Reum Han
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  2. Hye Ran Lee
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  3. Byeong-Bae Park
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  4. In Gyu Hwang
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  5. Sarah Park
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  6. Sang Cheol Lee
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  7. Kihyun Kim
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  8. Ho Yeong Lim
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  9. Young H. Ko
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  10. Sun Hee Kim
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  11. Won Seog Kim
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Corresponding author

Correspondence to Won Seog Kim.

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A-Reum Han and Hye Ran Lee contributed equally to this study.

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Han, AR., Lee, H.R., Park, BB. et al. Lymphoma-associated hemophagocytic syndrome: clinical features and treatment outcome. Ann Hematol 86, 493–498 (2007). https://doi.org/10.1007/s00277-007-0278-6

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  • DOI: https://doi.org/10.1007/s00277-007-0278-6

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