Can you survive mantle cell lymphoma

Fenske put a much more positive spin on more intensive treatment upfront with ASCT. The question is, does progression-free survival matter in mantle cell lymphoma? I argue that it does because relapse in patients with mantle cell lymphoma is no picnic.

Fenske, head of the section of bone marrow transplant and hematologic malignancies at the Medical College of Wisconsin in Milwaukee. As an example of the poor prognosis of relapsed or refractory mantle cell lymphoma patients Dr. Fenske cited a review he coauthored of 97 patients treated with ibrutinib Imbruvica.

Their median duration of response was 17 months and median progression-free survival was 15 months. Once ibrutinib treatment failure occurred their median overall survival was less than 3 months.

Hematol Oncol. Morphologically, both entities can present with small lymphoid cells. Marginal zone lymphoma is cyclin D1 negative and typically lacks CD5 expression.

The immunophenotypes of the two diseases are distinct. The blastoid variant of MCL has a high proliferation index, and the lymphoid cells may mimic the appearance of lymphoblasts seen in lymphoblastic lymphoma. There are no clear predisposing factors for mantle cell lymphoma. The disease affects significantly more men than women.

As with other subtypes of non-Hodgkin lymphoma, there may be an increased risk in patients who have a positive family history of lymphoma. Patients with mantle cell lymphoma should have a complete blood count with differential, and an evaluation of renal and liver function. For patients with a high burden of disease or blastoid variant MCL, a uric acid should be measured to determine the risk of tumor lysis syndrome. Hepatitis B serologies should be obtained in all patients who will receive therapy with rituximab, given the risk of viral reactivation associated with chemoimmunotherapy.

Routine sampling of cerebral spinal fluid is not typically indicated in the absence of clinical signs or symptoms, though patients with the blastoid variant are at higher risk of involvement of the central nervous system. Computed tomography CT scans of chest, abdomen and pelvis should be obtained as part of the staging evaluation. Positron emission tomography is also a useful imaging modality in this disease.

MCL is nearly uniformly fluorodeoxyglucose FDG avid and may highlight areas of disease, including the gastrointestinal tract, which may not be evident on CT scans. Evaluation of the gastrointestional tract GI with upper and lower endoscopies may be useful to fully stage patients. Patients with MCL do not typically require urgent therapy for bulky or symptomatic disease.

Rarely, patients with involvement of the GI tract may present with intussusception or bowel obstruction. In addition, patients may occasionally present with splenic rupture requiring emergent surgery. The blastoid variant is more likely to present with rapidly progressive disease. Steroids may temporize symptoms until more definitive therapy can be initiated. Because MCL is a rare disease, there are few randomized studies to guide initial therapy and clinical trial participation is encouraged.

A subset of patients, typically those with low volume, asymptomatic disease who often present with disease circulating in the peripheral blood and splenomegaly, those with a low Ki fraction or with mantle zone histology on pathologic review, may have a more indolent course. These patients may be candidates for observation, as demonstrated by a study from Cornell, which examined the role of observation in selected patients and showed no adverse impact on overall survival.

Fewer than half of patients, however, achieve a complete remission and the time to treatment failure is disappointing, at less than 2 years.

For younger patients without significant co-morbidities, more aggressive approaches are typically advocated. The combination, however, is highly myelosuppressive, and infectious complications are common. When the regimen was administered in the cooperative group setting by the Southwest Oncology Group, approximately half of patients received all planned therapy and median progression free and overall survival were 4.

An alternative approach to the initial therapy of MCL employs consolidation of patients in first remission, using high dose chemotherapy with autologous stem cell rescue. The median progression free survival was significantly longer in the ASCT group at 29, versus 17 months without a difference in overall survival. Both progression free survival and overall survival were superior in the latter arm.

Bendamustine plus rituximab is a highly active regimen and the preliminary results comparing this regimen to RCHOP, revealed a benefit in progression free survival in the bendamustine arm. In addition, there was a benefit in terms of progression free survival in those patients receiving rituximab maintenance.

Bendamustine is a highly active agent in this setting and may be combined with rituximab. 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.

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.

Your doctor might use your prognostic score to help decide on the most appropriate treatment for you. In around 1 in 10 people, mantle cell lymphoma grows slowly and causes few or no symptoms. Under a microscope, it has features of a low-grade lymphoma. These people might not need treatment for a long time, sometimes years. In most people, mantle cell lymphoma is fast-growing and treatment needs to start straightaway.

First-line treatment is usually successful at putting your lymphoma into remission shrinking the lymphoma or getting rid of it completely but the lymphoma almost always comes back within months or years, and needs more treatment. In the last few years, several new treatments have become available that have improved the outlook for many people with mantle cell lymphoma. Your medical team are best placed to advise you on your specific outlook based on your individual circumstances.

They can use the results of your tests and other factors for example, your age and physical fitness to help choose the best treatment for you. They are usually measured 5 or 10 years or more after treatment, so they only tell you how people did in the past. Treatment options for mantle cell lymphoma have improved a lot in recent years and those people might not have received the same treatment as you. Many people do not find survival statistics helpful because of this variability.

This involves having regular check-ups with your medical team to monitor your health and to see how the lymphoma is affecting you. You do not have treatment for the lymphoma unless it starts causing significant health problems. Active monitoring means that while you are well, you avoid the side effects of treatment for as long as possible. Treatment is still available when you need it, but this might not be for many months or, rarely, years.

Treating you before the lymphoma is causing problems does not make you live longer. If you develop troublesome symptoms, your lymph nodes become very large, or the lymphoma starts to affect your organs or blood counts, your medical team are likely to recommend treatment. The treatment options are usually the same as for fast-growing mantle cell lymphoma. The treatment your medical team recommends for you depends on the stage of your lymphoma and the signs and symptoms you have.

Most people have advanced stage mantle cell lymphoma when they are diagnosed. If you have early-stage mantle cell lymphoma, you might be offered radiotherapy to the affected area. This can be very successful, leading to long-lasting responses. Occasionally, it can cure the lymphoma, although in many people, it comes back and needs more treatment. The exact combination of drugs chemotherapy regimen your team recommends depends on the results of your tests and your individual circumstances.

You have a scan when you finish your treatment to see how you have responded. This is usually a CT scan. If you had lymphoma in your bone marrow before you started treatment, you might have another bone marrow biopsy. You might also have other tests.

Your doctor uses the results of the scan and other tests, if needed, to see if you are in remission your lymphoma has shrunk or disappeared completely or if you need further treatment. When you are in remission after treatment, you have regular follow-up appointments.

These are to:. At each appointment, your doctor examines you and asks if you have any concerns or symptoms. You might have blood tests. You are unlikely to have a scan unless you have new or worsening symptoms that could be a sign of your lymphoma coming back. How long lymphoma stays under control in remission after successful treatment varies from person to person. At some point, mantle cell lymphoma usually relapses in most people.

The treatment at this stage depends on:. Mantle cell lymphoma can relapse several times and you might have different treatments each time. Refractory lymphoma is usually treated in a similar way to relapsed lymphoma. Scientists are testing many different targeted treatments in clinical trials for mantle cell lymphoma, including some treatments that are already approved for other types of lymphoma.