Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2017 Jul 14;8(35):59977-59985.
doi: 10.18632/oncotarget.19230. eCollection 2017 Aug 29.

A systematic review of malignancy-associated hemophagocytic lymphohistiocytosis that needs more attentions

Hongluan Wang #  1   2   3 , Lixia Xiong #  1   2 , Weiping Tang  1   2 , Ying Zhou  2 , Fei Li  1
Affiliations
Review

A systematic review of malignancy-associated hemophagocytic lymphohistiocytosis that needs more attentions

Hongluan Wang et al. Oncotarget. .

Abstract

As an infrequent but potentially life-threatening hyperinflammatory syndrome, hemophagocytic lymphohistiocytosis (HLH) is clinically characterized with prolonged fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hyperferritinemia and hemophagocytosis in bone marrow, liver, spleen or lymph nodes. Malignancy-associated HLH (M-HLH), one type of acquired HLH, usually presents variable overlaps of symptoms with other types of HLH, thus resulting in higher incidence of misdiagnosis and mortality. In recent years, with the increasing awareness to this disease, the diagnosis and management of HLH have gained more and more attention, and improvements have been made accordingly. As a result, the survival of patients is greatly prolonged. However, there is still no consensus on the diagnostic criteria and treatment strategies due to lack of large samples or prospective clinical trials. In order to improve recognition and diagnosis, and provide guidance regarding the treatment of M-HLH, the Study Group in HLH Subtypes of the Histiocyte Society has developed consensus recommendations for the diagnosis and management of M-HLH in 2015. In the present article, we summarized and discussed some updated understandings in M-HLH.

Keywords: hemophagocytic lymphohistiocytosis; hemophagocytic syndrome; lymphoma; malignancy.

PubMed Disclaimer

Conflict of interest statement

CONFLICTS OF INTEREST The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1. The tumor types of malignancy-associated HLH
See this image and copyright information in PMC

References

    1. Chandrakasan S, Filipovich AH. Hemophagocytic lymphohistiocytosis: advances in pathophysiology, diagnosis, and treatment. J Pediatr. 2013;163:1253–1259. - PubMed
    1. Zhang K, Jordan MB, Marsh RA, Johnson JA, Kissell D, Meller J, Villanueva J, Risma KA, Wei Q, Klein PS, Filipovich AH. Hypomorphic mutations in PRF1, MUNC13-4, and STXBP2 are associated with adult-onset familial HLH. Blood. 2011;118:5794–5798. - PMC - PubMed
    1. Li F, Yang Y, Jin F, Dehoedt C, Rao J, Zhou Y, Li P, Yang G, Wang M, Zhang R, Yang Y. Clinical characteristics and prognostic factors of adult hemophagocytic syndrome patients: a retrospective study of increasing awareness of a disease from a single-center in China. Orphanet J Rare Dis. 2015;10:20. - PMC - PubMed
    1. Janka GE, Lehmberg K. Hemophagocytic syndromes—an update. Blood Rev. 2014;28:135–142. - PubMed
    1. Arico M, Danesino C, Pende D, Moretta L. Pathogenesis of haemophagocytic lymphohistiocytosis. Br J Haematol. 2001;114:761–69. - PubMed