title: Clinical features of adult patients with secondary hemophagocytic lymphohistiocytosis from causes other than lymphoma: an analysis of treatment outcome and prognostic factors.
abstract: Although hemophagocytic syndrome (HS) featuring secondary hemophagocytic lymphohistiocytosis (HLH) has a grave prognosis, little is known about the natural course of the disease. Patients who showed the clinical features of HLH as well as tissue-proven hemophagocytosis when seen at Asan Medical Center between 1999 and 2010 were included in this analysis. Patients with proven lymphoma were excluded. The median age of our 23 study patients was 49 years. Epstein-Barr virus was suspected to have caused HS in 16 (70%) patients and hepatitis A virus in one patient. Twenty-two patients were treated, 13 according to the HLH protocol and nine using immunosuppressive agents such as corticosteroid and/or cyclosporine. Five patients undertook allogeneic hematopoietic cell transplantation (HCT) during their treatment-dependent relapse (n = 4) or responsive status (n = 1). After the median follow-up of 180 days, 17 (74%) died and six (26%) were alive. The median time from initial presentation until death was 41 days among those patients who died. The serum fibrinogen level ≥166 mg/dL determined at the initial visit was significantly associated with the survival time according to univariate analysis. The low histiocyte proportion in bone marrow and early initiation of treatment tended to correlate with a favorable outcome. On multivariate analysis, serum fibrinogen ≥166 mg/dL (hazard ratio, 0.175, P = 0.018) was an independent clinical factor for determining the patient survival time. Despite appropriate patient management, the outcome of HS featuring HLH was grave. The serum fibrinogen level at the initial presentation was significant, and selected patients obtained some benefit from allogeneic HCT.
authors.affiliation: Department of Hematology, Asan Medical Center, University of Ulsan College of Medicine, Songpa-gu, Seoul, Republic of Korea.
authors.forename: Han-Seung
authors.initials: HS
authors.lastname: Park
authors.forename: Dae-Young
authors.initials: DY
authors.lastname: Kim
authors.forename: Je-Hwan
authors.initials: JH
authors.lastname: Lee
authors.forename: Jung-Hee
authors.initials: JH
authors.lastname: Lee
authors.forename: Sung-Doo
authors.initials: SD
authors.lastname: Kim
authors.forename: Young-Hun
authors.initials: YH
authors.lastname: Park
authors.forename: Jae Seok
authors.initials: JS
authors.lastname: Lee
authors.forename: Bo Youn
authors.initials: BY
authors.lastname: Kim
authors.forename: Mijin
authors.initials: M
authors.lastname: Jeon
authors.forename: Young-Ah
authors.initials: YA
authors.lastname: Kang
authors.forename: Young-Shin
authors.initials: YS
authors.lastname: Lee
authors.forename: Miee
authors.initials: M
authors.lastname: Seol
authors.forename: Yeon-Joo
authors.initials: YJ
authors.lastname: Lee
authors.forename: Young-Suk
authors.initials: YS
authors.lastname: Lim
authors.forename: Seongsoo
authors.initials: S
authors.lastname: Jang
authors.forename: Chan-Jeoung
authors.initials: CJ
authors.lastname: Park
authors.forename: Hyun-Sook
authors.initials: HS
authors.lastname: Chi
authors.forename: Kyoo-Hyung
authors.initials: KH
authors.lastname: Lee
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000453:epidemiology; D005260:Female; D004854:Herpesvirus 4, Human / Q000502:physiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis* / Q000209:etiology* / Q000628:therapy*; D008223:Lymphoma / Q000150:complications; D008297:Male; D008875:Middle Aged; D036102:Peripheral Blood Stem Cell Transplantation; D011379:Prognosis; D012307:Risk Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents