title: Richter Syndrome With Plasmablastic Lymphoma at Primary Diagnosis: A Case Report With a Review of the Literature.
abstract: Richter syndrome (RS) is considered as the rare development of an aggressive lymphoid malignancy in a preexisting small lymphocytic lymphoma/chronic lymphocytic leukemia. The most common aggressive lymphoma developing in this setting is diffuse large B-cell lymphoma, but classical Hodgkin lymphoma and other much rarer entities such as prolymphocytic lymphoma and dendritic cell sarcoma are also described, most frequently in the progression of the disease over time. A clonal relation between the 2 neoplastic proliferations can be frequently found, whereas clonally unrelated cases are commonly considered as independent tumors, probably due to a variable combination of multiple causes, responsible independently for the 2 neoplasms. RS with plasmablastic lymphoma is reported very rarely, during the clinical course of the small lymphocytic lymphoma/chronic lymphocytic leukemia. Herein, an unusual case of RS with the coexistence of plasmablastic lymphoma and B-small lymphocytic lymphoma in the same lymph node at the time of first diagnosis is described.
authors: *Pathology Unit, Seconda Università degli Studi di Napol †Pathology Unit ‡Hematology Unit, Istituto Nazionale dei Tumori "Pascale", Naples, Italy.
authors: Andrea
authors: A
authors: Ronchi
authors: Laura
authors: L
authors: Marra
authors: Ferdinando
authors: F
authors: Frigeri
authors: Gerardo
authors: G
authors: Botti
authors: Renato
authors: R
authors: Franco
authors: Annarosaria
authors: A
authors: De Chiara
mesh_terms: D006801:Humans; D008297:Male; D008875:Middle Aged; D000069293:Plasmablastic Lymphoma / Q000150:complications*