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Germinotropic lymphoproliferative disorder: a systematic review.

  • Zanelli, Magda1
  • Zizzo, Maurizio2, 3
  • Bisagni, Alessandra4
  • Froio, Elisabetta4
  • De Marco, Loredana4
  • Valli, Riccardo4
  • Filosa, Alessandra5
  • Luminari, Stefano6
  • Martino, Giovanni7
  • Massaro, Fulvio6
  • Fratoni, Stefano8
  • Ascani, Stefano9
  • 1 Pathology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy. [email protected] , (Italy)
  • 2 Surgical Oncology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy. , (Italy)
  • 3 Clinical and Experimental Medicine PhD Program, University of Modena and Reggio Emilia, Modena, Italy. , (Italy)
  • 4 Pathology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy. , (Italy)
  • 5 Pathology Unit, Area Vasta 5 (Ascoli Piceno-San Benedetto del Tronto), Ascoli Piceno, Italy. , (Italy)
  • 6 Hematology, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy. , (Italy)
  • 7 Hematology Unit, CREO, Azienda Ospedaliera di Perugia, University of Perugia, Perugia, Italy. , (Italy)
  • 8 Pathology Unit, St. Eugenio Hospital, Rome, Italy. , (Italy)
  • 9 Pathology Unit, Azienda Ospedaliera Santa Maria di Terni, University of Perugia, Terni, Italy. , (Italy)
Published Article
Annals of Hematology
Publication Date
Apr 19, 2020
DOI: 10.1007/s00277-020-04024-3
PMID: 32307569


Germinotropic lymphoproliferative disorder is a rare and rather enigmatic novel entity with distinctive clinicopathological features, one of which is the typical co-infection by Human herpesvirus 8 and Epstein-Barr virus. Human herpesvirus 8 is a lymphotropic virus detected in Kaposi sarcoma, multicentric Castleman disease, primary effusion lymphoma, Human herpesvirus 8-positive diffuse large B cell lymphoma not otherwise specified, and germinotropic lymphoproliferative disorder. Co-infection by Human herpesvirus 8 and Epstein-Barr virus is identified only in two lymphoproliferative diseases: germinotropic lymphoproliferative disorder and primary effusion lymphoma, which are otherwise diseases with totally different clinical presentations and outcomes. Unlike primary effusion lymphoma mostly occurring in immunocompromised individuals and following an aggressive course, germinotropic lymphoproliferative disorder usually presents with single or multiple lymphadenopathy affecting mainly immunocompetent individuals and mostly follows an indolent course. Based on the PRISMA guidelines, we carried out a systematic search on PubMed/MEDLINE, Web of Science, Scopus, EMBASE, and Cochrane Library using the search terms "germinotropic" and "lymphoproliferative disorder." Current scientific literature reports just 19 cases of germinotropic lymphoproliferative disorder. The purpose of our systematic review is to improve our understanding of the disease, focusing on epidemiology, clinical presentation, pathological features, treatment, and outcome. In addition, we discuss the differential diagnosis with the other Human herpesvirus 8-related lymphoproliferative diseases as currently recognized in the World Health Organization classification, adding a focus on lymphoproliferative disorders showing overlapping features.

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