General Principles

Peripheral T-cell lymphomas are rare; therefore, the best treatment approaches have not yet been established. The treatment paradigms that are used to treat T-cell lymphomas have been adapted from the more established management pathways of the more common B-cell lymphomas. More research is needed to create specialized, effective and hopefully, curative protocols for PTCL. Therefore, it’s very important to consider enrollment and participation in clinical trials at any stage of PTCL treatment (either at initial diagnosis, or at the time of relapse).

At the time of diagnosis, it is critical to establish whether you or your family member—as a patient—can tolerate the intensive chemotherapy regimens that are generally needed to induce complete remission (the complete disappearance of visible tumors on your CT-Scans) and potentially cure your lymphoma for good; or if such treatments would be too detrimental to your health due to other illnesses or debilitations and milder, “disease-controlling/stabilizing” treatments are more appropriate. The reason that this is important to establish is that the success of any cancer treatment is a combination of both, effective therapy and tolerability of treatments. This determination is not a simple one and takes into account a lot of factors (i.e., age, other diseases—heart disease, chronic kidney disease, diabetes, etc.). Your doctor will carefully evaluate your history and guide you through the decision process.  It is important to mention that aggressive treatments also place a burden on family and caregivers, since patients will require a lot of support after returning home from having received treatments. Multiple support groups and resources are available to help you through this process as well; please be sure to ask your doctor and social worker about these services.

If your doctor determines you can withstand intensive treatment, then typically a combination of several chemotherapy agents are used for initial treatment. The most commonly used regimens are CHOP, CHOEP, EPOCH, Hyper-CVAD and a few others. These are mostly administered in an outpatient setting in specialized medical facilities. Treatment proceeds in a cyclical manner with cycles repeated every 2-3 weeks. In general, 6-8 cycles of therapy will be delivered if your doctor determines that your lymphoma responds to the treatment.

In select cases, you might be offered an additional and more intense treatment called bone marrow transplantation. The intent of this procedure is to improve your chances to permanently eliminate the residual lymphoma cells left behind after initial chemotherapy. The benefit of bone marrow transplantation in first remission is controversial, and the decision is made on a case-by-case, individualized basis. Given the intensity of the bone marrow transplant procedure, not all patients are candidates to receive this treatment. As a general rule, specialists at academic centers that perform these types of treatments make the determination and decision regarding bone marrow transplantation.

If it happens that your lymphoma returns after the initial treatment that led to remission (called a ‘relapse’) or did not shrink completely with the first combination regimen, your doctor will discuss alternative treatments with you that are typically referred to as “salvage therapy.” The principles of deciding which type of salvage therapy you should receive is similar to those described above. If your doctor feels that you can tolerate even more aggressive chemotherapy regimens than the ones described above, you will be offered one of the established, intensive protocols. The most commonly used combinations are ICE, DHAP or ESHAP, as well as a few others; your doctor will determine the specific regimen. The intent of this treatment is to induce the best possible response (ideally complete remission) and proceed with one of the types of bone marrow transplantation (autologous or allogeneic). In this setting, it is not controversial; undergoing bone marrow transplantation is the only known way that your lymphoma can be cured.

If your doctor determines that aggressive salvage protocols, including transplant, are not suitable for your situation—either because of tolerance, or because the lymphoma did not respond to the first salvage treatment—an alternative approach can be pursued. Thanks to recent advances in research and clinical trials, several new agents have become available, specifically for relapsed or refractory T-cell lymphomas. Although it happens at a slightly lower rate than the intensive protocols, these new agents are capable of inducing remission, including complete remission. In general, the side effects of these agents are much lower than the above-described combinations. In addition, they do not seem to have the cumulative toxicity (worsening of side effects with multiple treatments) that is common with traditional chemotherapy. Because of this last feature, these new agents can be given for prolonged periods of time to those patients in whom the lymphoma responded to such treatment and, therefore, control their lymphoma for a long period of time (sometimes for years). It is important to understand that these prolonged treatments are unlikely to lead to cure, but can control your lymphoma and as a result improve your quality of life without having to endure significant side effects and toxicities. This approach is sometimes called palliative intent treatment, and for many patients, provides a way to live with their lymphoma and remain active in everyday life. In other words, one step short from a cure—including T-cell lymphomas—is transforming it into a livable condition for as long as possible (recall that the same principle applies to many non-cancerous conditions, such as diabetes, heart disease and other chronic, incurable illnesses).