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Case Reports in Hematology
Volume 2016, Article ID 4703608, 7 pages
Case Report

TAFRO Syndrome Associated with EBV and Successful Triple Therapy Treatment: Case Report and Review of the Literature

1Department of Medicine, Rhode Island Hospital and Warren Alpert School of Medicine, Brown University, Providence, RI, USA
2Department of Hematology and Oncology, Rhode Island Hospital, Providence, RI, USA
3Department of Pathology, Rhode Island Hospital, Providence, RI, USA

Received 15 July 2016; Accepted 25 August 2016

Academic Editor: Simon Davidson

Copyright © 2016 Malorie Simons et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


TAFRO syndrome is a rare constellation of symptoms: thrombocytopenia, anasarca, reticulin fibrosis of the bone marrow, renal dysfunction, and organomegaly. Its pathogenesis involves an excessive and inappropriate cytokine storm, most notably from IL-6, causing multiorgan failure; however, its etiology is undetermined. Starting in 2012, TAFRO syndrome was first identified in Japan as an atypical variant of Castleman’s disease. Previous reports include various different treatment protocols with inconsistent survival outcomes. Here we report the first known American, EBV positive but HIV and HHV-8 negative, male with TAFRO syndrome. He was successfully treated with an unusual three-drug regimen including tocilizumab, etoposide, and rituximab. We review the literature of TAFRO syndrome, discuss its possible viral etiology, and propose an original treatment regimen.