According to an article from journals.lww.com, the treatment of mantle cell lymphoma, a rare type of blood cancer, has historically been a significant medical challenge. However, recent developments in the treatment of the disease over the last few years have given mantle cell lymphoma patients a better chance for long term survival. For years the standard approach for this cancer involved an intense chemotherapy regimen that sometimes included immunotherapies as well. This was then followed with stem cell transplant. However, the introduction of new targeted therapies has begun to supersede the earlier standard.
About Mantle Cell Lymphoma (MCL)
Mantle cell lymphoma is a rare type of non-Hodgkin’s lymphoma. There are only about 15,000 patients in the US. This blood cancer affects B-cells, a type of white blood cell. The risk factors for mantle cell lymphoma are not particularly well known; however, acquired genetic mutations in the affected cells are what eventually causes them to become malignant. Most patients are diagnosed in their 60s. In many cases, the disease is not diagnosed until it has reached an advanced stage. Symptoms include fever, night sweats, enlarged spleen and lymph nodes, and weight loss. Treatment options include immunotherapy, chemotherapy, and targeted therapies. This cancer often relapses after treatment with chemotherapy. Prognosis is difficult to predict; the five year survival rate is 50 percent, but this figure improves to 70 percent with limited-stage disease. To learn more about mantle cell lymphoma, click here.
Some form of combination therapy is the most frequently tested regimen for mantle cell lymphoma. The Bruton’s tyrosine kinase inhibitor ibrutinib, which was first introduced in 2013, was tested in combination with the monoclonal antibody rituximab. Studies of this approach have produced impressive results with the majority of patients achieving a complete and long lasting response. This approach also minimizes exposure to chemotherapy.
Another combination that is undergoing evaluation is ibrutinib and venetoclax, which is an inhibitor of the BCL-2 protein. Results so far suggest that adverse events may be more frequent with this approach, but if the efficacy results are promising, it could be viable treatment option for some patients.
Another combination that has been tried is ibrutinib plus umbralisib, which is an investigational inhibitor of PI3K delta. This treatment didn’t appear to have a major advantage when compared to ibrutinib alone, but the researchers concluded by saying that further testing would be necessary to confirm if this therapy was useful.
CAR-T Cell Therapy
CAR-T cell therapy has also entered early phase 1 trials as a treatment for the disease. The overall response rate in this study was 78 percent. Although some serious adverse side effects did appear, the authors concluded that the approach displayed effective clinical activity in patients with refractory/relapsed disease and acceptable toxicity.
These ongoing trials for mantle cell lymphoma have the potential to discover a valuable new treatment regimen that could provide substantial improvements in survival and quality of life for patients with this rare cancer.