To Characterize and Compare Clinical, Pathological and Molecular Features Clinical Trial
Official title:
EBV-associated Diffuse Large B Cell Lymphoma NOS in Non Immunocompromised Patients : a French Retrospective Case Study
The aim of this study is to characterize and compare clinical, pathological (histology, immunophenotype) and molecular features of EBV-related GZL and DLBCL: do they belong to the same entity or to two different subtypes of EBV-related lymphoma ?
Recent progresses in the understanding of lymphoma biology allowed the identification of new
categories in the WHO classification of lymphoid neoplasm. Among these new entities, grey
zone lymphoma (GZL) between classical Hodgkin lymphoma and diffuse large B cell lymphoma
entered the classification as a provisional entity in 2008 and Diffuse large B cell lymphoma
(DLBCL) EBV-associated not otherwise specified in patient without history of immunodepression
in 2016. These entities need however to be further characterized as no clear pathological
definition and distinction between EBV related GZL and EBV related DLBCL are proposed in the
literature.
After primary infection that occurs in almost 80% of young adult population in western
country, EBV latency state settles in a small portion of B cells. The virus is known to
promote B-cells proliferation and transformation through complex mechanisms.
EBV-associated lymphoproliferative disorders have already been largely studied in
immunocompromised host (human immunodeficiency virus, congenital immunodeficiency,
immunosuppressor and post-transplant lymphoproliferative disorder, e.g.), but its role is
largely unknown in patients with no history of immunodeficiency. Whether these patients arbor
an underlying type of immune deficiency has not yet been established, even though
immunosenecence in elderly or immaturity of the immune system in children has been postulated
in those population.
Based on former definition, EBV-associated lymphoproliferation were usually the apanage of
classical Hodgkin lymphoma (cHL) or immunodeficiency-associated lymphoproliferative
disorders. However, as published recently, several different histological patterns with
intermediate features between primary mediastinal B cell lymphoma (PMBCL) and classical
Hodgkin lymphoma (cHL), namely grey zone lymphoma (GZL), but also diffuse large B cell
lymphoma (DLBCL) were described to be EBV-associated. On the clinical point of view, patients
with GZL are young and have a poor prognosis. Data regarding EBV+ DLBCL are discordant and
further studies are warranted, even though initial report concluded to a poor prognosis for
these patients.
;