Lymphoma-associated hemophagocytic syndrome: clinical features and treatment outcome |
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Authors: | 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 Kim |
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Institution: | Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea. |
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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.
A-Reum Han and Hye Ran Lee contributed equally to this study. |
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Keywords: | Hemophagocytic syndrome Lymphoma-associated hemophagocytic syndrome |
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