Successful experience of treatment of a patient with generalized non-GCB- DLBCL using the R-mNHL-BFM-90 protocol with lenalidomide: case report and review of literature

Габеева, Н Г; Звонков, Е Е; Koroleva, D.; Chukavina, Marina; Obukhova, T N; Kovrigina, А М

doi:10.26442/terarkh201890796-101

Cited by 2 publications

(1 citation statement)

References 29 publications

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“…In our clinic, the use of the intensive block program R-mNHL-BFM-90 resulted in the improved treatment of a group of DLBCL patients with unfavorable prognosis factors [ 22 ]. A retrospective evaluation of the results showed that in most cases of treatment failure, the tumor belonged to the postgerminal immunohistochemical variant of DLBCL (non-germinal center B-cell (non-GCB)-DLBCL), a molecular analog of the ABC-DLBCL subtype [ 23 ].…”

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confidence: 99%

Non-GCB Diffuse Large B-Cell Lymphoma With an Atypical Disease Course: A Case Report and Clinical Exome Analysis

et al. 2022

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Diffuse large B-cell lymphoma (DLBCL) is the most common lymphoid tumor among other non-Hodgkin lymphomas (30-40% of all cases). This type of lymphoma is characterized by significant differences in treatment response and the heterogeneity of clinical traits. Approximately 60% of patients are cured using standard chemotherapy (CT), while in 10-15% of cases, the tumor is characterized by an extremely aggressive course and resistance to even the most high-dose programs with autologous stem cell transplantation (auto-SCT). The activated B-cell (ABC) subtype of DLBCL is characterized by poor prognosis. Here, we describe a clinical case of diffuse ABC-DLBCL with an atypical disease course. Complete remission was achieved after four courses of CT, followed by autologous hematopoietic stem cell transplantation (auto-HSCT). However, early relapse occurred 2 months after the completion of treatment. According to the results of cytogenetic studies, significant chromosome breakdowns were observed. Exome sequencing allowed for the detection of several novel mutations that affect components of the NOTCH2 and NF-κB signaling pathways, a number of epigenetic regulators (KMT2D, CREBBP, EP300, ARID1A, MEF2B), as well as members of the immunoglobulin superfamily (CD58 and CD70). Whether these mutations were the result of therapy or were originally present in the lymphoid tumor remains unclear. Nevertheless, the introduction of genomic technologies into clinical practice is important for making a diagnosis and developing a DLBCL treatment regimen with the use of targeted drugs.

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Section: Introductionmentioning

confidence: 99%

Non-GCB Diffuse Large B-Cell Lymphoma With an Atypical Disease Course: A Case Report and Clinical Exome Analysis

et al. 2022

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CHEMOTHERAPY ACCORDING TO THE R-mNHL-BFM-90 PROTOCOL IN COMBINATION WITH LENALIDOMIDE AS THE FIRST LINE THERAPY IN PATIENTS WITH MUM1-POSITIVE DIFFUSIVE LARGE B-CELL LYMPHOMA AND FOLLICULAR LYMPHOMA GRADE 3B

Габеева¹,

Koroleva²,

Смольянинова³

et al. 2019

Gematologiâ i transfuziologiâ

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Introduction.Diffuse large B-cell lymphoma of postgerminal origin (ABC-DLBCL) and follicular lymphoma grade 3B (FL3B) are characterised by an aggressive course and resistance to chemotherapy (CT). Both diseases are characterised by the activation of genes of the post-terminal stage of B-cell differentiation and high expression of the MUM1 transcriptional protein. Lenalidomide in combination with R-CHOP improved the results of treatment in patients with ABC-DLBCL; however, about 40 % of them remain resistant to the therapy.Aim.The aim of the study was to evaluate the efficacy and toxicity of the R-mNHL-BFM-90 protocol with lenalidomide (R2-mNHL-BFM-90), as well as to analyse possible causes of CT resistance in patients with ABC-DLBCL and FL3B.Patients and methods.Over the period from October 2016 to December 2018, 8 patients with MUM1-positive DLBCL and FL3B were included in the research. All patients underwent a cytogenetic study of tumour samples. A mutational status of the TP53 gene was determined by Sanger sequencing.Results.Patients received combination chemotherapy according to the R2-mNHL-BFM-90 protocol with lenalidomide at a dose of 25 mg/day, from the 1st to the 10th day of each course. Autologous hematopoietic stem cell transplantation was performed as a consolidation in three patients. After the end of the chemotherapy, a complete remission of the disease was achieved in all patients. Relapse developed in 1 patient with a mutation in the TP53 gene. With a median follow-up period of 11 months (1–23), event-free survival was 87 %.Conclusions. The R2-mNHL-BFM-90 protocol has demonstrated a high efficacy and acceptable toxicity in patients with ABC-DLBCL and FL3B. The presence of a mutation in the TP53 gene is established to be an extremely unfavourable prognostic factor even provided intensive treatment protocols, thus requiring the development of alternative approaches to the management of such patients.

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Successful experience of treatment of a patient with generalized non-GCB- DLBCL using the R-mNHL-BFM-90 protocol with lenalidomide: case report and review of literature

Cited by 2 publications

References 29 publications

Non-GCB Diffuse Large B-Cell Lymphoma With an Atypical Disease Course: A Case Report and Clinical Exome Analysis

Non-GCB Diffuse Large B-Cell Lymphoma With an Atypical Disease Course: A Case Report and Clinical Exome Analysis

CHEMOTHERAPY ACCORDING TO THE R-mNHL-BFM-90 PROTOCOL IN COMBINATION WITH LENALIDOMIDE AS THE FIRST LINE THERAPY IN PATIENTS WITH MUM1-POSITIVE DIFFUSIVE LARGE B-CELL LYMPHOMA AND FOLLICULAR LYMPHOMA GRADE 3B

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