Treatment of Epstein-Barr virus induced hemophagocytic lymphohistiocytosis with anti-CD20 therapy: A two-year follow-up

Bijit Kumar Kundu ； Deepak Rath

《International Journal of Case Reports and Images (IJCRI)》 9卷1期 (2018/06) Pp. 25-32

https://doi.org/10.5348/ijcri-201803-CR-10872

摘要

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.

關鍵字

Epstein-Barr virus ； Hemophagocytic lymphohistiocytosis ； Panniculitis ； Rituximab