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

Abstract

Background Plasmablastic lymphoma (PBL) is a rare, aggressive B-cell lymphoma with poor prognosis usually found in the oral cavity of HIV-positive patients. Chronic lymphocytic leukemia (CLL) is an indolent B-cell lymphoma with a variable clinical course. Transformation of CLL to PBL as Richter's syndrome is rare while coexistence of CLL and PBL at diagnosis is even rarer. Case Report We describe a case of a male immunocompetent patient with an ileum-cecum valve mass and a soft tissue mass at the left humerus with histologic evidence of PBL with coexistence of CLL in the bone marrow and peripheral blood. Amputation of the patient's left arm was inevitable, and the patient was started on bortezomib and dexamethasone. However, prolonged hospitalization was complicated by aspiration pneumonia, and the patient passed away. Conclusions No standard of care exists for patients with PBL, and prognosis remains dismal. Concomitant presentation of hematological malignancies becomes increasingly recognized, and further insight is needed in order to delineate whether they originate from the same clone or from different ones.

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.…”

“…R-CHOP was selected for the present case, similar to a previous case of concurrent DLBCL and PBL that showed recurrence of DLBCL after 4 years [9]. Other cases of PBL co-existing with CLL/SLL were also refractory to R-CHOP and/or vincristine, adriamycin, and dexamethasone (VAD) treatment (Table 1, cases 1-5) [10][11][12][13][14]. Basically, standard treatment for PBL has not been established due to the rarity of the disease and its aggressive clinical course.…”

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.…”

Role of Chronic Lymphocytic Leukemia (CLL)-Derived Exosomes in Tumor Progression and Survival

“…As previously mentioned, CLL is a very heterogenous B cell malignancy with a wide range of behaviors from patient to patient. Since 1928, a very aggressive and invasive form of lymphoma derived from CLL, named Richter's syndrome (RS) has been described [76][77][78][79]. A recent work has analyzed the circulating EVs in both CLL and RS patients and discovered high levels of miR-19b as predictor of the evolution of drug-resistant CLL toward RS, resulting in down-regulation of TP53 and up-regulation of MKI67, as mechanism of tumor cell proliferation, survival and invasion [80].…”