title: Lymphoma-associated hemophagocytic syndrome: clinical features and treatment outcome.
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.
authors.affiliation: Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea.
authors.forename: A-Reum
authors.initials: AR
authors.lastname: Han
authors.forename: Hye Ran
authors.initials: HR
authors.lastname: Lee
authors.forename: Byeong-Bae
authors.initials: BB
authors.lastname: Park
authors.forename: In Gyu
authors.initials: IG
authors.lastname: Hwang
authors.forename: Sarah
authors.initials: S
authors.lastname: Park
authors.forename: Sang Cheol
authors.initials: SC
authors.lastname: Lee
authors.forename: Kihyun
authors.initials: K
authors.lastname: Kim
authors.forename: Ho Yeong
authors.initials: HY
authors.lastname: Lim
authors.forename: Young H
authors.initials: YH
authors.lastname: Ko
authors.forename: Sun Hee
authors.initials: SH
authors.lastname: Kim
authors.forename: Won Seog
authors.initials: WS
authors.lastname: Kim
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D002648:Child; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D008223:Lymphoma / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies
chemical_list: D000970:Antineoplastic Agents