Case Report
 
Treatment of Epstein–Barr virus induced hemophagocytic lymphohistiocytosis with anti-CD20 therapy: A two-year follow-up
Bijit Kumar Kundu1, Deepak Rath2
1MD, Associate Professor, Rheumatology Clinic, Department of Medicine, PGIMER & Dr RML Hospital, New Delhi, India
2MD, Postdoctoral Trainee, Department of Rheumatology, IPGMER & SSKM Hospital, Kolkata, India

Article ID: Z01201801CR10872BK
doi:10.5348/ijcri-201803-CR-10872

Corresponding Author:
Dr. Deepak Rath,
Post-Doctoral Trainee, Department of Rheumatology,
IPGMER and SSKM Hospital, AJC Bose Road,
Kolkata – 700020, India

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How to cite this article
Kundu BK, Rath D. Treatment of Epstein–Barr virus induced hemophagocytic lymphohistiocytosis with anti-CD20 therapy: A two-year follow-up. Int J Case Rep Images 2018;9(1):25–32.


ABSTRACT

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is well known to the rheumatologist as a devastating complication of a rheumatologic disease, being one of the few rheumatologic emergencies. Its treatment is usually started on the basis of presumption without waiting for confirmatory evidence. The diagnosis of HLH is usually missed when it appears in any of its many and varied chronic manifestation. It can be a great mimic with symptoms pertaining to any organ system of the human body. Once diagnosed it is imperative to find the trigger in order to prevent recurrences.
Case Report: We present a case where HLH caused by Epstein–Barr virus (EBV) infection, presenting as recurring panniculitis; which continued for years before being diagnosed. Treatment with additional anti–CD20 therapy prevented further recurrences.
Conclusion: We recommend integrating anti–CD20 therapy as part of the treatment regimen of HLH triggered by EBV.

Keywords: Epstein–Barr virus, Hemophagocytic lymphohistiocytosis, Panniculitis, Rituximab



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Author Contributions
Bijit Kumar Kundu – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published Deepak Rath – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Guarantor of Submission
The corresponding author is the guarantor of submission.
Source of Support
None
Conflict of Interest
Authors declare no conflict of interest.
Copyright
© 2018 Bijit Kumar Kundu et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.