Systematic literature review of the global burden of illness of mantle cell lymphoma

Monga, Neerav; Garside, Jamie; Quigley, Joan; Hudson, M.; O′Donovan, Peter; O'Rourke, John; Tapprich, Christoph; Parisi, Lori; Davids, Matthew S.; Tam, Constantine S.

doi:10.1080/03007995.2020.1742101

Cited by 5 publications

(10 citation statements)

References 40 publications

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“…The less common variant of diffuse FL has a five-year survival rate of around 50% [ 2 ]. MCL are generally more aggressive, and most studies, even with intensive treatment regimens, failed to demonstrate a plateau in the survival curve on long-term follow-up [ 3 ]. Despite rationally applied molecularly driven combined chemo-immunotherapeutic approaches, prognosis remains dismal, especially for the relapsed/refractory (R/R) aggressive NHLs calling for novel treatment strategies.…”

Section: Introductionmentioning

confidence: 99%

PAK4 and NAMPT as Novel Therapeutic Targets in Diffuse Large B-Cell Lymphoma, Follicular Lymphoma, and Mantle Cell Lymphoma

Khan

Uddin

Balasubramanian

et al. 2021

Cancers

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Diffuse large B-cell lymphoma (DLBCL), grade 3b follicular lymphoma (FL), and mantle cell lymphoma (MCL) are aggressive non-Hodgkin’s lymphomas (NHL). Cure rates are suboptimal and novel treatment strategies are needed to improve outcomes. Here, we show that p21-activated kinase 4 (PAK4) and nicotinamide phosphoribosyl transferase (NAMPT) is critical for lymphoma subsistence. Dual targeting of PAK4-NAMPT by the Phase I small molecule KPT-9274 suppressed cell proliferation in DLBCL, FL, and MCL. Growth inhibition was concurrent with apoptosis induction alongside activation of pro-apoptotic proteins and reduced pro-survival markers. We observed NAD suppression, ATP reduction, and consequent cellular metabolic collapse in lymphoma cells due to KPT-9274 treatment. KPT-9274 in combination with standard-of-care chemotherapeutics led to superior inhibition of cell proliferation. In vivo, KPT-9274 could markedly suppress the growth of WSU-DLCL2 (DLBCL), Z-138, and JeKo-1 (MCL) sub-cutaneous xenografts, and a remarkable increase in host life span was shown, with a 50% cure of a systemic WSU-FSCCL (FL) model. Residual tumor analysis confirmed a reduction in total and phosphorylated PAK4 and activation of the pro-apoptotic cascade. This study, using various preclinical experimental models, demonstrates the therapeutic potential of targeting PAK4-NAMPT in DLBCL, FL, and MCL. The orally bioavailable, safe, and efficacious PAK4-NAMPT dual inhibitor KPT-9274 warrants further clinical investigation.

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Section: Introductionmentioning

confidence: 99%

PAK4 and NAMPT as Novel Therapeutic Targets in Diffuse Large B-Cell Lymphoma, Follicular Lymphoma, and Mantle Cell Lymphoma

Khan

Uddin

Balasubramanian

et al. 2021

Cancers

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“…Different from other chronic mature B-cell malignancies, MCL seems to be in Mexican Mestizos as frequent as in Caucasians: Carballo-Zárate et al 5 have shown that MCL represents in México 6.7% of all B cell NHL, a figure similar to that informed from the United States, which is around 5% 1,2 . As far as the prognosis is concerned, we found that outcomes are substantially better than those informed in the literature both in Caucasian individuals 3 and Taiwanese patients 10 , and could suggest that MCL has a more benign course in Mexican mestizos.…”

Section: Discussionmentioning

confidence: 80%

“…Mantle cell lymphoma (MCL) is a rare and aggressive disease which accounts for approximately 5% of all B-cell non-Hodgkin's lymphomas (NHL) 1 . The incidence of MCL is higher in non-Hispanic Whites in the United States 2,3 .…”

Section: Introductionmentioning

confidence: 99%

Mantle cell lymphoma may have a different clinical course in Mexican Mestizos: Real-world data from a single center

Córdova-Ramírez

Sánchez-Valledor

Colón‐Otero

et al. 2021

RIC

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Background: The biology of some hematological diseases varies among different populations. No previous studies have evaluated the clinical behavior of mantle cell lymphoma (MCL) in México. Objective and Methods: This is a retrospective review of MCL cases seen in Mexico from January 2003 to June 2020. A total of 12 cases were identified. Results: There were nine males and three females; median age was 56 years. Eight patients had a high MCL international prognostic index score, one was intermediate, and three were low. Five patients had circulating malignant monoclonal cells. Initial treatment included rituximab, cyclophosphamide, daunorubicin, vincristine, and prednisone (R-CHOP) and CHOP. Subsequent treatment included hematopoietic stem cell transplantation in five patients; two were given maintenance therapy. Splenectomy was done in four patients. Median overall survival (OS) for all the patients has not been reached and exceeds 162 mos: OS at 162 mos was 56%. Achieving a complete remission (CR) after the first treatment was a significant prognostic factor, with a median OS exceeding 141 mos in patients achieving CR, and 16 mos among those not achieving CR (p = 0.0006). Conclusion: Some of MCL patients in Mexico have an indolent clinical course, particularly patients who achieve a CR to initial treatment and who undergo splenectomy. (REV INVEST CLIN. [AHEAD OF PRINT]

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“…Mantle cell lymphoma (MCL) is an infrequent subtype of non-Hodgkin lymphoma (NHL) that accounts for approximately 5 to 7% of lymphoid malignancies in Western Europe, 1 but its incidence seems to be increasing over time. 2 It is an incurable disease with a median age at diagnosis of 68 years, 3 more common in men than in women (ratio around 3:1). 1 , 2 …”

Section: Introductionmentioning

confidence: 99%

“… 2 It is an incurable disease with a median age at diagnosis of 68 years, 3 more common in men than in women (ratio around 3:1). 1 , 2 …”

Section: Introductionmentioning

confidence: 99%

Real-World Evidence of Relapsed/Refractory Mantle Cell Lymphoma Patients and Treatments: A Systematic Review

Sancho,

Sorigué,

Rubio-Azpeitia

2024

JBM

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Introduction Mantle cell lymphoma (MCL) is an incurable disease with an aggressive clinical course, and most patients eventually relapse after chemotherapy. Targeted therapies developed for relapsed/refractory MCL have been approved based on clinical trial data. However, real-world setting data are scarce and scattered. Areas Covered This systematic review aimed to collect, synthesize, and describe the characteristics and treatment outcomes of patients with relapsed/refractory MCL after receiving a second or subsequent line of therapy in the real-world setting. Expert Opinion R/R MCL is clinically and biologically heterogeneous and still represents a therapeutic challenge, with high-risk and early relapsed patients remaining an unmet medical need. This systematic review is limited by the quality of the available data and the difficulty of comparing outcomes in R/R MCL due to the heterogeneity of the disease, but the results suggest that covalent BTKis should be positioned as second-line therapy, followed by CAR T-cells in BTK-i-relapsed patients. Chemo-free and combination therapies with established chemoimmunotherapy backbones in the relapsed and front-line settings have been recently developed, and front-line options are being improved to move targeted and cellular therapies to earlier lines, including front-line therapy, in elderly and younger fit patients. In the upcoming years, many new targeted agents will play an important role and will be incorporated to the routine practice as their sequence, and outcomes in unselected patients are determined.

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Systematic literature review of the global burden of illness of mantle cell lymphoma

Cited by 5 publications

References 40 publications

PAK4 and NAMPT as Novel Therapeutic Targets in Diffuse Large B-Cell Lymphoma, Follicular Lymphoma, and Mantle Cell Lymphoma

PAK4 and NAMPT as Novel Therapeutic Targets in Diffuse Large B-Cell Lymphoma, Follicular Lymphoma, and Mantle Cell Lymphoma

Mantle cell lymphoma may have a different clinical course in Mexican Mestizos: Real-world data from a single center

Real-World Evidence of Relapsed/Refractory Mantle Cell Lymphoma Patients and Treatments: A Systematic Review

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