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.

Concurrent intestinal plasmablastic lymphoma and diffuse large B-cell lymphoma with a clonal relationship: a case report and literature review

“…The concomitant occurrence of PBL with other types of lymphoma is extremely rare. Review of the English literature revealed only six case reports in which the histopathological findings of two lymphomas were described [9][10][11][12][13][14]. The clinicopathological features of these previous cases and the present case are summarized in Table 1.…”

Plasmablastic Lymphoma with Coexistence of Chronic Lymphocytic Leukemia in an Immunocompetent Patient: A Case Report and Mini-Review

“…The patient was originally treated with R-CHOP to progressive disease, then with bortezomib, ifosfamide, etoposide, carboplatin, and radiotherapy again to progressive disease, and later was given one infusion of brentuximab vedotin to which he showed dramatic response, but did not receive any further treatment due to recurrent gastrointestinal bleeding. Ronchi et al [ 16 ] described an unusual case of RS with the coexistence of PBL and SLL in the same lymph node at the time of the first diagnosis. Using needle cores from the two different areas of the lymph node and by employing immunoglobulin gene rearrangement studies, the authors showed that the two neoplasms were clonally related.…”

“…Coexistence of PBL with CLL has been described thus far in two patients [ 15 , 16 ]. Holderness et al described the first case of PBL arising in an HIV-negative, previously untreated CLL patient [ 15 ] who responded to brentuximab vedotin treatment as third-line treatment, while Ronchi et al [ 16 ] most recently reported the simultaneous coexistence of PBL and CLL in the same lymph node in another HIV-negative, previously untreated CLL patient. Both patients had very poor overall survival (OS).…”

Molecular characterization and clonal evolution in Richter transformation: Insights from a case of plasmablastic lymphoma (RT‐PBL) arising from chronic lymphocytic leukaemia (CLL) and review of the literature

“…None of the patients had a known history of immunodeficiency. Three cases had both CLL and PBL diagnosed simultaneously [2, 10, 11], whereas for the remaining 12 cases, the time between CLL diagnosis and PBL diagnosis varied from 14 to 132 months. Four patients had received prior treatment with Ibrutinib for a duration of 18–96 months before developing plasmablastic lymphoma.…”

“…A review of the literature on PBL arising in the context of CLL yielded 15 cases [2,[7][8][9][10][11][12][13][14][15][16], the findings of which are summarized in and a 17p13.1 deletion, along with a TP53 mutation. 14 In two cases, FISH analysis was performed for BCL2, BCL6, CCND1, MYC and TP53 abnormalities in both the CLL and PBL.…”