scholarly journals Chronic Lymphocytic Leukemia with Divergent Richter’s Transformation into a Clonally Related Classical Hodgkin’s and Plasmablastic Lymphoma: A Case Report

2020 ◽  
Vol 13 (1) ◽  
pp. 120-129 ◽  
Author(s):  
Gorana Gasljevic ◽  
Mateja Grat ◽  
Veronika Kloboves Prevodnik ◽  
Biljana Grcar Kuzmanov ◽  
Barbara Gazic ◽  
...  

Chronic lymphocytic leukemia (CLL) typically pursues a prolonged course. Its transformation into a more aggressive lymphoma occurs in 2–8% of all patients. Most commonly, diffuse large B-cell lymphoma develops. Transformation into a classical Hodgkin’s lymphoma (cHL) occurs in <1%. Plasmablastic transformation has been only rarely reported. Cases of synchronous divergent transformation of CLL into a composite lymphoma are exceedingly rare. We describe the unique occurrence of the transformation of a long-standing CLL into a synchronous clonally related cHL as well as plasmablastic lymphoma (PBL) in an 85-year-old female patient. After 10 years of asymptomatic CLL, our patient was treated with a rituximab-chlorambucil scheme in combination with pegfilgrastim for recurrent infections and the development of B symptoms. Five cycles (of six planned) were administrated with no adverse effects. After the fifth cycle, lymphadenopathy with pronounced B symptoms appeared. Histology showed the presence of cHL in the lymph node, while the bone marrow was infiltrated by PBL. Our patient died in sepsis not receiving further specific oncologic treatment due to her poor general condition. Additional cytogenetic and molecular studies showed that this was a case of mutated CLL with trisomies of chromosomes 12, 3, and 18 (a rare specific +12 plus other-non+19 CLL subgroup). The presence of trisomy 12 has also been proved in plasmablasts and in cHL cells.

Author(s):  
Harsh Shah ◽  
Deborah Stephens ◽  
John Seymour ◽  
Kami Maddocks

The introduction of novel targeted agents and immunotherapeutic modalities into the treatment of B-cell lymphomas has drastically shifted the treatment landscape. In diffuse large B-cell lymphoma, recent approvals of CAR T-cell therapy, the antibody-drug conjugate polatuzumab, and the anti-CD19 monoclonal antibody tafasitamab have provided efficacious options for patients with relapsed and refractory disease. These immunotherapies attempt to harness power from the patient’s own immune system to eradicate lymphoma. In chronic lymphocytic leukemia, oral targeted kinase inhibitors such as ibrutinib and acalabrutinib (Bruton tyrosine kinase inhibitors) and venetoclax (BCL2 inhibitor) are now favored over chemoimmunotherapy for upfront treatment because of improved progression-free survival across all subgroups (including high-risk subgroups such as unmutated immunoglobulin variable heavy chain and chromosome 17p deletion). In indolent lymphomas, several PI3K inhibitors are approved for treatment of relapsed disease. However, uptake of these agents has been limited because of toxicity concerns. Combination of lenalidomide and rituximab has been a safe and effective immune modality for patients with refractory indolent lymphomas; it is currently being used as a backbone to bring other targeted agents such as tazemetostat (EZH2 inhibitor) into earlier lines of treatment. In this article, we will review novel commercially available agents in the treatment of relapsed/refractory diffuse large B-cell lymphoma, treatment-naïve chronic lymphocytic leukemia, and relapsed/refractory indolent lymphomas. We will evaluate clinical trials that led to their approval and will provide an outlook into the future novel agents currently under investigation in B-cell malignancies.


2019 ◽  
Vol 37 (15_suppl) ◽  
pp. e18198-e18198
Author(s):  
Mark Price ◽  
Arliene Ravelo ◽  
Maria Sae-Hau ◽  
Peggy Ann Torney ◽  
Victor Gonzalez ◽  
...  

e18198 Background: Evaluations of patients’ (pt) burden and priorities are increasingly important as novel treatments are developed. We aimed to better understand pt-reported disease burden in chronic lymphocytic leukemia (CLL), diffuse large B-cell lymphoma (DLBCL), and follicular lymphoma (FL). Methods: We developed a survey to understand pt disease burden, designed in consultation with medical experts, pt advocacy organizations, and survey scientists. Concept elicitation and cognitive pretesting were conducted. The survey was administered electronically to pts who had received either initial or subsequent treatment within the past year and consisted of categorical Likert options that quantified the impact of disease on physical function, sleep, cognition, work, emotional health, and quality of life (QoL). Results: The Leukemia & Lymphoma Society and the Lymphoma Research Foundation recruited 424 pts: 309 with CLL, 59 with DLBCL, and 69 with FL; 51% were female. Mean age was 66 (range: 22–95). Results suggest that pts experience substantial disease burden across all surveyed subtypes. The most noteworthy results are highlighted in the following table that indicates the percentage of pts who agreed or strongly agreed to statements about disease burden. Additionally, large proportions of pts worried about their disease returning or getting worse (72% of pts with CLL, 83% of pts with DLBCL, and 79% of pts with FL), indicating high impact regardless of the indolent or aggressive nature of their disease. Most pts indicated that delaying disease progression was important to them (96% of pts with CLL, 96% of pts with DLBCL, and 92% of pts with FL). Employment status changed for 31% of pts who were working full or part time or on contract because of their diagnosis and treatment. Conclusions: Pts with CLL, DLBCL, and FL report experiencing substantial disease burden. Data from studies focusing on pt-reported disease burden can be used for education of the clinical community to address the key concerns of pts.[Table: see text]


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