Haema 2017; 8(1): 95-108
by Maria K. Angelopoulou, Pantelis Tsirkinidis, Theodoros Vasilakopoulos
National and Kapodistrian University of Athens, Department of Hematology and Bone Marrow Transplantation, Athens, Greece
Abstract
Within the aggressive B-cell lymphomas, some extremely rare entities have been described, including DLBCL associated with chronic inflammation, lymphomatoid granulomatosis, intravascular large B cell lymphoma, ALK-positive large B cell lymphoma, plasmablastic lymphoma, large cell lymphoma arising in HHV8-associated multicentric Castleman disease and primary effusion lymphoma. The vast majority of these lymphomas present with extranodal disease. At least four of these entities (ALKpositive large B cell lymphoma, plasmablastic lymphoma, large cell lymphoma arising in HHV8-associated multicentric Castleman disease and primary effusion lymphoma) are characterized by plasmablastic morphology and immunophenotype of the neoplastic cells. A common feature of these distinct entities is the presence of acquired immunodeficiency (e.g. HIV infection) with the exception of intravascular and ALK+ large B cell lymphomas. The immunodeficiency status leads to loss of immunosurveillance and allows two gamma herpes virus, namely EBV and HHV-8 to promote lymphomagenesis. More specifically, EBV relates pathogenetically with DLBCL associated with chronic inflammation, lymphomatoid granulomatosis and plasmablastic lymphoma, whereas HHV-8 is linked to large cell lymphoma arising in HHV8-associated multicentric Castleman disease and primary effusion lymphoma. These aggressive rare B cell lymphomas are characterized by poor prognosis.