Germinotropic lymphoproliferative disorder: a systematic review.

Magda Zanelli, Maurizio Zizzo, Alessandra Bisagni, Elisabetta Froio, Loredana De Marco, Riccardo Valli, Alessandra Filosa, Stefano Luminari, Giovanni Martino, Fulvio Massaro, Stefano Fratoni, Stefano Ascani

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)2243-2253
Number of pages11
JournalAnnals of Hematology
Issue number10
Publication statusPublished - Oct 2020


  • Adult
  • Aged
  • Aged, 80 and over
  • Antineoplastic Combined Chemotherapy Protocols/therapeutic use
  • Coinfection/virology
  • Combined Modality Therapy
  • Diagnosis, Differential
  • Disease Progression
  • Female
  • Germinal Center/pathology
  • Herpesviridae Infections/diagnosis
  • Herpesvirus 4, Human/isolation & purification
  • Herpesvirus 8, Human/isolation & purification
  • Humans
  • Immunocompetence
  • Lymph Nodes/pathology
  • Lymphoma, Large B-Cell, Diffuse/virology
  • Lymphoma, Non-Hodgkin/virology
  • Lymphoma, Primary Effusion/diagnosis
  • Lymphoproliferative Disorders/diagnosis
  • Male
  • Middle Aged
  • Treatment Outcome


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