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

Hongluan Wang, Lixia Xiong, Weiping Tang, Ying Zhou and Fei Li _

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Oncotarget. 2017; 8:59977-59985. https://doi.org/10.18632/oncotarget.19230

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Hongluan Wang1,2,3,*, Lixia Xiong1,2,*, Weiping Tang1,2, Ying Zhou2 and Fei Li1

1Department of Hematology, The First Affiliated Hospital of Nanchang University, Nanchang, Jiangxi 330006, China

2Medical College, Nanchang University, Nanchang, Jiangxi 330006, China

3Department of Respiratory, Jiangxi Provincial People’s Hospital, Nanchang, Jiangxi 330006, China

*These authors contributed equally to this work

Correspondence to:

Fei Li, email: [email protected]

Keywords: hemophagocytic lymphohistiocytosis, hemophagocytic syndrome, malignancy, lymphoma

Received: May 17, 2017     Accepted: June 29, 2017     Published: July 14, 2017


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.

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