Non-EBV infection-associated hemophagocytic lymphohistiocytosis: a distinct subgroup where pathogen-directed therapy is essential and favorable outcomes are expected

Abstract

EBV is the most prevalent cause of infection-associated hemophagocytic lymphohistiocytosis (IAHLH), non-EBV IAHLH is observed clinically but less documented. We conducted a retrospective research enrolled 36 cases from 1/1/2015 to 31/12/2019. Intriguingly, 92% cases were immunocompetent individuals prior to the onset of HLH. Clinically, the cardinal features were prolonged high fever, splenomegaly and hemophagocytosis. Bicytopenia occurred in most patients, besides, liver dysfunction was characterized by increased transaminase, bilirubin, alkaline phosphatase (ALP), gamma-glutamyl transpeptidase (γ-GGT) and lactate dehydrogenase (LDH). Immunomodulatory agents should be added to control the overwhelming inflammatory storm without delay. Once a certain pathogen was identified as the causative factor of HLH, cytotoxic agents were withdrawn, specific pathogen-directed treatment was initiated. Further, glucocorticoids were tapered off when a stable state of HLH was achieved. After treatment, about 70% patients were fully recovered without relapse. All in all, non-EBV IAHLH is a special group of HLH with admirable outcome.

Keywords:

• Non-EBV
• infection
• hemophagocytic lymphohistiocytosis
• treatment

Acknowledgements

The authors would like to thank all the doctors and nurses in the Department of Hematology in Beijing Friendship Hospital, Capital Medical University. We also thank the haematologists, radiation, laboratory, pathologists and all other participating department for the sustained scientific collaboration.

Disclosure statement

The authors declared that they have no commercial, proprietary, financial interest or any potential conflicts in the producers or companies described in this manuscript. All authors have submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest. Besides, the information has never been presented in any meeting previously.

Additional information

Funding

This work was supported by grants from the National Natural Science Foundation of China (grant number: 81871633), the Beijing Natural Science Foundation (grant number: 7181003) and Beijing Municipal Administration of Hospital Incubating Program (Code: PX2018003).