Haemophagocytic lymphohistiocytosis in adults: a multicentre case series over 7 years

Br J Haematol. 2016 Feb;172(3):412-9. doi: 10.1111/bjh.13837. Epub 2015 Nov 5.


Haemophagocytic lymphohistiocytosis (HLH) is a syndrome of uncontrolled immune activation that has gained increasing attention over the past decade. Although classically known as a familial disorder of children caused by mutations that affect cytotoxic T-cell function, an acquired form of HLH in adults is now widely recognized. This is often seen in the setting of malignancy, infection or rheumatological disorders. We performed a retrospective review across 3 tertiary care centres and identified 68 adults with HLH. The average age was 53 years (range 18-77 years) and 43 were male (63%). Underlying disorders included malignancy in 33 patients (49%), infection in 22 (33%), autoimmune disease in 19 (28%) and idiopathic HLH in 15 (22%). Patients were treated with disease-specific therapy and immunomodulatory agents. After a median follow-up of 32·2 months, 46 patients had died (69%). The median overall survival was 4 months (95% CI: 0·0-10·2 months). Patients with malignancy had a worse prognosis compared to those without (median survival 2·8 months versus 10·7 months, P = 0·007). HLH is a devastating disorder with a high mortality. Further research is needed to improve treatment and outcomes.

Keywords: ferritin; haemophagocytic lymphohistiocytosis; haemophagocytosis; immune activation; macrophage activation syndrome.

Publication types

  • Multicenter Study

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Autoimmune Diseases / complications
  • Female
  • Follow-Up Studies
  • Humans
  • Infections / complications
  • Lymphohistiocytosis, Hemophagocytic / drug therapy*
  • Lymphohistiocytosis, Hemophagocytic / etiology
  • Male
  • Middle Aged
  • Neoplasms / complications
  • Prognosis
  • Retrospective Studies
  • Survival Analysis
  • Treatment Outcome
  • Young Adult