Back to Journals » Cancer Management and Research » Volume 11

Clinical characteristics of extranodal NK/T-cell lymphoma-associated hemophagocytic lymphohistiocytosis

Authors Liu YZ, Bi LQ, Chang GL, Guo Y, Sun S

Received 13 August 2018

Accepted for publication 29 November 2018

Published 23 January 2019 Volume 2019:11 Pages 997—1002

DOI https://doi.org/10.2147/CMAR.S183784

Checked for plagiarism Yes

Review by Single-blind

Peer reviewers approved by Dr Colin Mak

Peer reviewer comments 3

Editor who approved publication: Dr Ahmet Emre Eskazan


Yi-Zhen Liu,1,2,* Lan-Qing Bi,3,* Gui-Lin Chang,4 Ye Guo,5 Si Sun1,2

1Department of Medical Oncology, Fudan University Shanghai Cancer Center, Shanghai 200032, China; 2Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200032, China; 3Department of Medical Oncology, Suzhou Municipal Hospital, The Affiliated Suzhou Hospital of Nanjing Medical University, Suzhou 215000, China; 4Department of Geratology, Fudan University Shanghai Zhongshan Hospital, Shanghai Medical College, Fudan University, Shanghai 200032, China; 5Department of Medical Oncology, Shanghai East Hospital, Tongji University School of Medicine, Shanghai 200120, China

*These authors contributed equally to this work

Background: Extranodal natural killer (NK) / T cell lymphoma is a subtype of non-Hodgkin’s lymphoma (NHL) that usually has an aggressive clinical course. It is the predominant trigger of lymphoma-associated hemophagocytic syndrome (LAHS), which is highly lethal and with extremely poor prognosis. This study is aiming to characterize the associated clinical features and prognostic factors of the disease.
Patients and methods: Twenty-eight patients with extranodal NK/T cell lymphoma associated hemophagocytic lymphohistiocytosis (HLH) were retrospectively analyzed. The clinical records were collected, and the associations between clinical or laboratory parameters and overall survival (OS) were assessed.
Results: The most frequently clinical characteristics were fever (96.4%), and splenomegaly (81.5%). Concerning the laboratory findings, the most common features were hyperferritinemia (91.7%), grade III/IV thrombocytopenia (64.3%), hypertriglyceridemia (48%), severe anemia (46.4%), hypofibrinogenemia (45%), and grade III/IV neutropenia (32.1%). The interval between the diagnosis of NK/T LAHS and death / last contact was between 4 to 701 days with the median interval of 15 days. We found that higher serum lactic dehydrogenase (LDH) at HLH, hypofibrinogenemia, and splenomegaly were significantly associated with worse survival (P=0.002, 0.003, 0.003). Furthermore, Eastern Cooperative Oncology Group (ECOG) score, extra-upper aerodigestive tract NK/T cell lymphoma (EUNKTL) and cutaneous involvement were risk factors of HLH.
Conclusion: Our data indicated that levels of LDH, fibrinogen, and presence of splenomegaly were prognostic factors of the disease. Higher ECOG scores, EUNKTL and cutaneous involvement were risk factors of NK/T LAHS. Additional independent, prospective clinical trials will be needed to explore optimal treatment.

Keywords: NK/T-cell lymphoma, hemophagocytic lymphohistiocytosis, risk factor, prognosis

Creative Commons License This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution - Non Commercial (unported, v3.0) License. By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms.

Download Article [PDF]  View Full Text [HTML][Machine readable]