Mantle Cell Lymphoma: Clinical Characteristics, Prevalence and Treatment Options

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Nevertheless, the favorable long-term survival outcome of early-stage MCL in our study, may point to the need for a treatment paradigm shift for these patients. Patients with early-stage MCL may be suitable for less intensive therapy, or a minimalist approach with abbreviated chemotherapy and radiation therapy to maximize disease control while limiting long-term toxicity.

Over the past few decades, the theme for lymphomas with long survival rates has been to de-escalate therapy. In one study, Hershman et al. Along the same effort of using fewer cycles of chemotherapy, a lower radiation dose has been recently used.

It is noteworthy that in our study, patients who received radiation therapy as a sole modality, in combined approach or for recurrence after relapse following chemotherapy had lower rates of failure at the original disease site. Our encouraging results suggest that in patients who are not candidate for doxorubicin-based chemotherapy due to underlying cardiac morbidity, radiation therapy alone should be strongly considered and can potentially cure two-thirds of the patients.

In our study, many characteristics were not associated with overall outcome, including the presence of extranodal or nodal disease, the number of involved sites, site at presentation, and importantly, type of management. This lack of association reflects a pattern common to low-grade lymphomas, in which we learned more than half a decade ago that outcomes were not affected by extent of initial therapy, ranging from intensive treatment including high-dose therapy and stem cell transplantation to simply observation, similar to 12 of the patients in our current study.

Can the prognosis of mantle cell lymphoma be predicted by si : Medicine

Future studies evaluating for these biomarkers in patients with limited stage MCL may further support its indolent nature. Because early-stage MCL is an infrequent presentation, we collected data from several institutions around the world to provide a more robust analysis; however, this is also a major shortcoming, as cases were not managed consistently, including extent of staging work-up, laboratory testing thus limiting our ability to determine the MIPI score 4 , and treatment selection biases.


Nevertheless, through this international collaborative effort, we were able to demonstrate a favorable long-term survival outcome in patients with early stage MCL, regardless of the type or intensity of the treatment. Because this was a retrospective multi-institutional study, its findings should still be regarded as preliminary, to guide future efforts to define a subgroup of MCLs that might have a different biologic etiology as well as different outcomes. Oxford University Press is a department of the University of Oxford. It furthers the University's objective of excellence in research, scholarship, and education by publishing worldwide.

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Sign In. Advanced Search. Article Navigation. Close mobile search navigation Article Navigation. Volume Article Contents. Statistical analysis. Oxford Academic. Google Scholar. Cite Citation. Permissions Icon Permissions. Abstract Background. Figure 1. Open in new tab Download slide. A Overall survival of all patients. B Overall-survival according to treatment modality. Figure 2.

Racial differences in mantle cell lymphoma in the United States

Mantle cell lymphoma. A proposal for unification of morphologic, immunologic, and molecular data. Search ADS. A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group. From centrocytic to mantle cell lymphoma: a clinicopathologic and molecular review of 3 decades. A clinical evaluation of the International Lymphoma Study Group classification of non-Hodgkin's lymphoma. A new prognostic index MIPI for patients with advanced-stage mantle cell lymphoma.

A clinical analysis of two indolent lymphoma entities: mantle cell lymphoma and marginal zone lymphoma including the mucosa-associated lymphoid tissue and monocytoid B-cell subcategories : a Southwest Oncology Group study. Genomic and gene expression profiling defines indolent forms of mantle cell lymphoma. Limited-stage mantle cell lymphoma: treatment outcomes at the Princess Margaret Hospital. Report of an international workshop to standardize response criteria for non-Hodgkin's lymphomas.

Doxorubicin, cardiac risk factors, and cardiac toxicity in elderly patients with diffuse B-cell non-Hodgkin's lymphoma. Radiation therapy is an effective modality in the treatment of mantle cell lymphoma, even in heavily pretreated patients. All rights reserved. For permissions, please email: journals. Issue Section:. Download all figures. View Metrics. Email alerts New issue alert. Advance article alerts. Article activity alert.

Receive exclusive offers and updates from Oxford Academic. More on this topic Central nervous system involvement in mantle cell lymphoma: clinical features, prognostic factors and outcomes from the European Mantle Cell Lymphoma Network. Current treatments and promising investigations in a multidisciplinary setting. Chemotherapy for squamous cell carcinoma of head and neck: The future is now. Related articles in Web of Science Google Scholar. Epstein - Barr virus infection in de novo diffuse large B-cell lymphoma in Jordan and Turkey.

Citing articles via Web of Science 3. Prediction of response to anti-PD-1 therapy in patients with non-small-cell lung cancer by electronic nose analysis of exhaled breath. Facilitating access to new therapeutic options through clinical trials: the vision of a regulator to reconcile innovation and safety. Looking for your next opportunity? In addition, second line chemotherapy regimens typically used in aggressive lymphomas, such as: RICE rituximab, ifosfamide, carboplatin, etoposide , RDAP rituximab, dexamethasone, cytarabine, Cisplatin , Rgem-ox rituximab, gemcitabine, oxaliplatin are also active regimens.

For younger patients with chemotherapy sensitive disease, reduced intensity allogeneic stem cell transplant may offer the possibility of long term disease control.

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Graft-versus host disease and infectious complications, however, may cause significant short and long term morbidity and mortality. Rasburicase should be administered in the rare event that a patient develops frank tumor lysis.

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In patients over 65 receiving aggressive chemoimmunotherapy regimens, white blood cell growth factors Neupogen or pegfilgrastim should be employed to reduce the rates of febrile neutropenia. In addition, patients receiving bendamustine are at increased risk for varicella zoster reactivation and patients receiving fludarabine are at risk for both Pneumocystis jiroveci pneumonia and zoster and these patients should receive appropriate prophylaxis.

Clinical Features, Prognosis and Treatment of Follicular Lymphoma

Mantle cell lymphoma is not curable with conventional chemoimmunotherapy. Overall, the median survival is approximately 6 to 7 years. For younger patients, aggressive chemotherapy with or without consolidation with high dose chemotherapy and stem cell rescue prolongs progression free survival, compared to standard therapy with a median of more than 5 years. The Mantle Cell Lymphoma International Prognostic Index is a strong predictor of outcome and has been validated in multiple studies.

In addition, the blastoid variant of MCL, which typically has a high Ki fraction has a clinically aggressive course and is also associated with a higher risk of relapse in the central nervous system CNS. There are a number of active chemotherapy agents for patients with relapsed disease, including ibrutinib, bortezomib, lenalidomide, and bendamustine, as well as combination regimens used in aggressive lymphomas. For younger patients without significant co-morbidities, patients whose disease responds to chemotherapy may be candidates for reduced intensity allogeneic stem cell transplantation.

Patients should receive high dose methotrexate or cytarabine based regimens which achieve adequate CNS penetration with or without intrathecal chemotherapy for leptomeningeal involvement.

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If a patient has a favorable response to therapy, stem cell transplantation should be considered. The majority of cases of MCL are thought to arise from pre-germinal center B-cells, that demonstrate rearranged heavy and light chain genes, typically without somatic hypermutation. A subset of cases, however, may be derived from B-cells which are antigen experienced as evidenced by somatic hypermutation.

Standard cyotogentics identify the t 11;14 translocation in approximately half of cases, but the vast majority of cases will be positive by fluorescence in situ hybridization. A very small subset of cases express cyclin D2, as opposed to cyclin D1, but overexpression of SOX11 is seen in both groups. Additional genetic events that inactivate the normal pathways of response to DNA are common. The majority of cases of the blastoid variant harbor additional cytogenetic abnormalities, with frequent mutations involving p In addition, abnormalities in genes involved in the cell cycle, such as p16, p17 and p27, are common.

Copy number changes have also been frequently identified in mantle cell lymphoma, particularly gains in 3q and 6p and losses of 13q. Mantle cell lymphoma frequently involves the gastrointestional tract. Patients undergoing routine colonoscopy are rarely found to have diffuse small polyps, which on pathologic examination demonstrate involvement by MCL.

Typically, patients with MCL present with asymptomatic, diffuse lymphadenopathy, and splenomegaly, but may also develop fevers, night sweats, or weight loss, particularly in patients with a high disease burden or with blastoid variant. Involvement of the CNS is very rare at presentation with MCL but is more common in patients with the blastoid variant.

Occasionally, patients will present in with a significant lymphocytosis with or without splenomegaly and lymphadenopathy mimicking the presentation of CLL. The immunophenotype CD20 bright with absent CD23 and flourescence in situ hybridization FISH cytogenetics demonstrating a t 11;14 are critical for establishing the diagnosis of MCL, though chromosomal abnormalities typical of CLL, such as deletions in13q and 17p may be identified in these cases. In this paper, the Ki fraction was identified as an independent predictor of survival in MCL. J Clin Oncol..

Despite high overall and molecular completes response rates, the median progression free survival was poor at This randomized study demonstrated a prolongation of progression free survival in patients who received autologous stem cell transplantation compared to interferon. This study demonstrates that in selected patients with MCL, deferred initial therapy is a reasonable therapeutic option. This paper is a comprehensive review of the biology of mantle cell lymphoma and examines rationale targeted approaches to therapy.

N Engl J Med.. All rights reserved. No sponsor or advertiser has participated in, approved or paid for the content provided by Decision Support in Medicine LLC. Login Register. Powered By Decision Support in Medicine. Jump to Section Mantle cell lymphoma What every physician needs to know: Are you sure your patient has mantle cell lymphoma?

What should you expect to find? Beware of other conditions that can mimic mantle cell lymphoma: Which individuals are most at risk for developing mantle cell lymphoma: What laboratory studies should you order to help make the diagnosis and how should you interpret the results? What imaging studies if any will be helpful in making or excluding the diagnosis of mantle cell lymphoma? If you decide the patient has mantle cell lymphoma, what therapies should you initiate immediately?

More definitive therapies? What other therapies are helpful for reducing complications? What should you tell the patient and the family about prognosis? What if scenarios. What if a patient relapses following high dose chemotherapy and autologous stem cell rescue? What if a patient develops disease involving the central nervous system?

Pathophysiology What other clinical manifestations may help me to diagnose mantle cell lymphoma? What other additional laboratory studies may be ordered?