title: Lymphoma-associated hemophagocytic lymphohistiocytosis: experience in adults from a single institution.
abstract: Lymphoma-associated hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal disease. Differences between B cell and T cell lymphoma-associated HLH remain unclear, specifically clinical characteristics and survival. We retrospectively analyzed 30 lymphoma-associated HLH patients from July 2004 to October 2012. Patients were divided into B cell (n = 13) and T cell (n = 17) lymphoma groups. Patients' age, performance status, presence of Epstein-Barr virus infection, international prognostic index, presence of disseminated intravascular coagulopathy, serum triglyceride, fibrinogen, and lactate dehydrogenase levels were not significantly different between B cell and T cell lymphoma groups. HLH was an indicator for treatment resistance in patients with B cell (p = 0.048), but not T cell (p = 0.217), lymphoma. Patients in the T cell lymphoma group, however, had higher serum ferritin levels than patients in the B cell lymphoma group (11,525.6 versus 3,790.6 ng/mL; p = 0.043). The median survival time for patients in the B cell and T cell lymphoma groups was 330 and 96 days, respectively. Although the difference was not statistically significant (p = 0.273), our results suggested a trend toward a better overall survival time in patients with B cell lymphoma. This survival advantage could be at least partially due to use of rituximab (p = 0.045) for the treatment of patients with B cell lymphoma. Our results also suggested that allogeneic hematopoietic stem cell transplantation could possibly provide survival benefits to T cell lymphoma-associated HLH by graft-versus-lymphoma effect.
authors.affiliation: Division of Hematology/Medical Oncology, Department of Medicine, Taichung Veterans General Hospital, 160, Section 3, Taichung Port Road, Taichung, 407, Taiwan, Republic of China.
authors.forename: Jui-Ting
authors.initials: JT
authors.lastname: Yu
authors.forename: Chen-Yu
authors.initials: CY
authors.lastname: Wang
authors.forename: Youngsen
authors.initials: Y
authors.lastname: Yang
authors.forename: Ren-Ching
authors.initials: RC
authors.lastname: Wang
authors.forename: Kuang-Hsi
authors.initials: KH
authors.lastname: Chang
authors.forename: Wen-Li
authors.initials: WL
authors.lastname: Hwang
authors.forename: Chieh-Lin Jerry
authors.initials: CL
authors.lastname: Teng
mesh_terms: D000328:Adult; D000368:Aged; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis* / Q000401:mortality* / Q000628:therapy; D016393:Lymphoma, B-Cell / Q000175:diagnosis* / Q000401:mortality* / Q000628:therapy; D016399:Lymphoma, T-Cell / Q000175:diagnosis* / Q000401:mortality* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D055815:Young Adult