Malignancy-associated hemophagocytic lymphohistiocytosis in adults: a retrospective population-based analysis from a single center

Abstract

A retrospective, population-based study was conducted to evaluate the incidence, clinical features, and outcome of malignancy-associated hemophagocytic lymphohistiocytosis (M-HLH) in adults. Between January 1996 and December 2009, eight out of 887 (0.9%) patients diagnosed with hematological malignancies developed aggressive M-HLH in an area inhabited by approximately 160 000 people. Thus the estimated annual incidence of M-HLH in adulthood was 0.36/100 000 individuals/year. The clinical course of M-HLH was aggressive in all patients. Six patients were treated with a modified HLH-94 protocol; three achieved remission (durable in one case) while the others did not respond and died within an average of 2.4 months (range 1.5–3.5) after M-HLH diagnosis. Infection complicating the course of M-HLH occurred in four (50%) patients, all of whom developed fulminant M-HLH and died. Although the small study population limits the results, the long observation period strengthens its value.

Keywords:

• Hemophagocytic lymphohistiocytosis
• hemophagocytic syndrome
• hemophagocytosis
• malignancy-associated
• malignancy

Acknowledgements

The authors are indebted to Professor Jan-Inge Henter for critical review of the manuscript, Mr. Erik Holmberg, regional Oncology Center in Gothenburg, for setting up the data on the incidence of hematological malignancies in northern Halland, and Steven Shev, MD, PhD and Nick Bolton, PhD for linguistic support.

Potential conflict of interest: Disclosure forms provided by the authors are available with the full text of this article at www.informahealthcare.com/lal.