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EBV Infection and Human Primary Immune Deficiencies

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Front. Immunol. | doi: 10.3389/fimmu.2018.00368


  • 1Department of Pediatrics, Dr. von Hauner Children’s Hospital, University Hospital, LMU Munich, Germany
  • 2Institute of Pathology, Faculty of Medicine, LMU Munich, Germany, Germany
  • 3Department of Radiology, University Hospital, LMU Munich, Germany, Germany
  • 4Pathology Unit, Department of Molecular and Translational Medicine, University of Brescia, Italy

EBV positive smooth muscle tumors constitute a very rare oncological entity. They usually develop in the context of secondary immunodeficiency caused by human immunodeficiency virus infection or immunosuppressive treatment after solid organ transplantation. However, in a small fraction of predominantly pediatric patients, EBV positive smooth muscle tumors may occur in patients with primary immunodeficiency disorders such as GATA2 and CARMIL2 deficiency. In secondary immunodeficiencies and when the underlying condition can not be cured, the treatment of EBV positive smooth muscle tumors is based on surgery in combination with antiretroviral and reduced or altered immunosuppressive pharmacotherapy, respectively. Importantly, without definitive reconstitution of cellular immunity, long term survival is poor. This is particularly relevant for patients with EBV smooth muscle tumors on the basis of primary immunodeficiency disorders. Recently allogeneic hematopoietic blood stem cell transplantation resulted in cure of immunodeficiency and EBV positive smooth muscle tumors in a GATA-2 deficient patient.
We propose that in the absence of secondary immunodeficiency disorders patients presenting with EBV positive smooth muscle tumors should be thoroughly evaluated for primary immunodeficiency disorders. Allogeneic hematopoietic stem cell transplantation should be taken into consideration, ideally in the setting of a prospective clinical trial.

Keywords: Epstein Barr virus, Smooth Muscle Tumor, Primary immunodeficiency disorder, secondary immunodeficiency disorder, allogeneic hematopoietic stem cell transplantation, CARMIL2, GATA2.

Received: 21 Nov 2017; Accepted: 09 Feb 2018.

Edited by:

Isabelle Meyts, KU Leuven, Belgium

Reviewed by:

Alexandra Freeman, National Institutes of Health (NIH), United States
Flore Rozenberg, Université Paris Descartes, France
Cindy S. Ma, Garvan Institute of Medical Research, Australia  

Copyright: © 2018 Magg, Schober, Walz, Ley-Zaporozhan, Facchetti, Klein and Hauck. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: MD, PhD. Fabian Hauck, Dr. von Hauner Children’s Hospital, University Hospital, LMU Munich, Department of Pediatrics, Munich, Germany,