Clinical, pathological and molecular features of plasmablastic lymphoma arising in the gastrointestinal tract: A review and reappraisal

Plasmablastic lymphoma (PBL) is a CD20-negative large B-cell lymphoma with a plasmacytic phenotype and a dismal prognosis, which has been defined as a distinct entity only in the 2008 WHO Classification of Haematopoietic and Lymphoid Tissue and confirmed in the 2017 Edition. Current knowledge of the biological, clinical and prognostic features of PBL is mostly limited, resulting in diagnostic issues, as well as in lack of standard of care and effective therapeutic options. PBL commonly affects the oral cavity of HIV-positive individuals, however the gastrointestinal (GI) tract is the most common extraoral site, and in this location most patients are HIV-negative. In this review, we focus on the clinical, morphological and prognostic features of PBL arising in the GI tract, in order to improve knowledge on this rare, but aggressive disease.