Lymphoma-associated hemophagocytic lymphohistiocytosis: experience in adults from a single institution

Ann Hematol. 2013 Nov;92(11):1529-36. doi: 10.1007/s00277-013-1784-3. Epub 2013 May 23.

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.

MeSH terms

  • Adult
  • Aged
  • Cohort Studies
  • Female
  • Follow-Up Studies
  • Humans
  • Lymphohistiocytosis, Hemophagocytic / diagnosis*
  • Lymphohistiocytosis, Hemophagocytic / mortality*
  • Lymphohistiocytosis, Hemophagocytic / therapy
  • Lymphoma, B-Cell / diagnosis*
  • Lymphoma, B-Cell / mortality*
  • Lymphoma, B-Cell / therapy
  • Lymphoma, T-Cell / diagnosis*
  • Lymphoma, T-Cell / mortality*
  • Lymphoma, T-Cell / therapy
  • Male
  • Middle Aged
  • Retrospective Studies
  • Young Adult