Abstract
Introduction: Mantle cell lymphoma (MCL) belongs to the mature B cell-lymphoproliferative disorders. It accounts for 6-8 % of adult lymphoma cases in Europe.
Diagnosis is based on histological examination of a lymph node or bone marrow. MCL is characterized by the translocation t (11; 14) (q13; q32), which is present in 95 % of cases.
The disease may be indolent, but typically presents with an aggressive clinical course. MCL is currently considered an incurable disease (allogeneic stem cell transplantation being the only potentially curative option) and often results in recurrent relapses or disease progression.
The prognosis of primary progressive (PP) disease, which occurs in 10 % patients, is higly unfavourable with estimated median overall survival being 1-2 years. Case report: The authors present a case of a patient with PP MCL diagnosed in May 2012.
A 67-year-old male was initially treated with R-CHOP alternating with R-ARA-C (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone/rituximab, cytosine- arabinoside). The disease progressed early after induction therapy in November 2012.
Subsequently, the patient was enrolled into a phase 2 clinical trial MCL-002-SPRINT and was randomized into lenalidomide treatment arm. This therapy was associated with severe myelotoxicity resulting in the dose reductions and interruptions of lenalidomide.
Eventually, lenalidomide treatment had to be permanently discontinued because of severe myelotoxicity (neutropenia) in December 2014. Although the median progression-free survival was 8.7 months in the lenalidomide of the SPRINT study, the partial response in this patient lasted 2 years despite modifications lenalidomide therapy.
Further progression of MCL was diagnosed in February 2015 and ibrutinib was chosen for the 3rd -line treatment. Despite initial partial remission, the disease progressed after 4 months of ibrutinib treatment in September 2015.
Combination of ARA-C and corticosteroids was used until bendamustine has approved by the patient's health insurance company. However, the disease progressed on bendamustine + rituximab regimen.
As all the therapeutic options were exhausted, the patient was started on palliative/symptomatic therapy in December 2015 and died of lymphoma progression after 3 years and 8 months from initial diagnosis. Conclusion: This case report highlights the importance of clinical trials for elderly patients with relapsed/refractory lymphoma as these trials offer new therapeutic approaches for those who have unsatisfactory results with currently available options.
Novel small molecules such as lenalidomide and ibrutinib provide new hope for prognosis improvement in patients with relapsed/ refractory MCL.