AUTHOR=Chang Yueh-Shih , Ye Jung-Jr , Cheng Tzu-Chien , Wen Ying-Hao , Huang Chi-Ying F. , Yeh Kun-Yun 

TITLE=Case report: Reactive Epstein–Barr virus-associated hemophagocytic lymphohistiocytosis and thrombosis with thrombocytopenia syndrome following SARS-CoV-2 vaccination and treated with intravenous immunoglobulin

JOURNAL=Frontiers in Hematology

VOLUME=1

YEAR=2022

URL=https://www.frontiersin.org/journals/hematology/articles/10.3389/frhem.2022.983424

DOI=10.3389/frhem.2022.983424

ISSN=2813-3935

ABSTRACT=<p>Viral reactivation was previously reported after severe acute respiratory syndrome coronavirus‐2 (SARS-CoV-2) infection but was seldom documented after SARS-CoV-2 vaccination, except varicella-zoster virus and cytomegalovirus. Here, we present a case of reactive Epstein–Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (HLH) and thrombosis with thrombocytopenia syndrome after receiving SARS-CoV-2 mRNA vaccination. Antiplatelet factor 4 antibody was detected, and the bone marrow study showed hemophagocytosis and was positive in the immunohistochemistry staining for EBV-encoded small nuclear RNAs and negative staining for CD3 and CD56 markers of small lymphocytes. The high percentage of CD38 <sup>high</sup>/HLA-DR<sup>+</sup> cells among CD8<sup>+</sup> T cells further confirmed HLH. After intravenous administration of immunoglobulin, the clinical symptoms, D-dimer level, fibrinogen, platelet count, EBV-DNA titer, and anti-PF4 level were all improved. Further investigation into the pathogenesis of vaccine-associated EBV reactivation, such as TNF-α, interleukin-1β (IL-1β), and interleukin-6 (IL-6), is warranted.</p>