TITLE

Lymphoma-associated hemophagocytic syndrome: clinical features and treatment outcome

AUTHOR(S)
A-Reum Han; Hye Lee; Byeong-Bae Park; In Hwang; Sarah Park; Sang Lee; Kihyun Kim; Ho Lim; Young Ko; Sun Kim; Won Kim
PUB. DATE
July 2007
SOURCE
Annals of Hematology;Jul2007, Vol. 86 Issue 7, p493
SOURCE TYPE
Academic Journal
DOC. TYPE
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.
ACCESSION #
25137992

 

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