Clinical Director, Lymhoma Inpatient Unit, Memorial Sloan-Kettering Cancer Center
What is Hodgkin lymphoma and how is it diagnosed?
Hodgkin lymphoma (HL) is a cancer of the immune system. It is unique in that it affects a somewhat younger patient population, with the median age being 28 years old. It is a relatively rare disease, affecting about 9,000 patients per year in the United States.
A lymphoma diagnosis is suspected when an individual has a lump, possibly felt in their neck, or has symptoms related to lymphoma such as fevers, night sweats, or weight loss. Occasionally, enlarged lymph nodes are detected incidentally when a patient undergoes a scan for another reason, leading their doctor to suspect lymphoma. The diagnosis of lymphoma, including Hodgkin lymphoma, requires a lymph node biopsy. Once a diagnosis is made we then determine where and to what extent it is located in the patient's body, a process called staging. This is done with a computed tomography (CT) scan and a positron emission tomography (PET) scan of the body.
What are the current treatment options for Hodgkin lymphoma?
The treatment of HL varies depending upon an individual’s stage at diagnosis. For patients who have stage I or II, or early stage disease, we consider giving a combination of chemotherapy plus radiation therapy although more recent studies are trying to reduce the use of radiation therapy to decrease the toxicity associated with treatment. Patients with advanced stage disease, or stage III or IV, are typically treated with chemotherapy alone. The most common chemotherapy regimen we use in the United States for HL is ABVD (adriamycin [doxorubicin], bleomycin, vinblastine, and dacarbazine). However, there are other regimens used in both early and advanced stage HL, particularly in parts of Europe where a regimen called escalated BEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone) is more commonly used.
The majority of patients are cured with initial treatment. However, those who do not respond well to initial treatment or whose disease returns still have a good chance of being cured with second-line treatment. Currently, the standard second-line treatment is an additional course of chemotherapy followed by consolidation with an autologous stem cell transplant (where the patient serves as his or her own donor for stem cells).
Current clinical trials are evaluating exciting new first-line and second-line treatment options for HL. Brentuximab vedotin, a drug that was approved in August 2011, has shown significant activity for patients who have relapsed after second-line treatment. Because of that, it is now being evaluated at every phase of treatment for HL. The newest class of drugs showing impressive activity in relapsed HL includes anti-PD-1 monoclonal antibodies, which work by activating a patient’s immune system to help attack their lymphoma. Upcoming studies will be evaluating strategies to incorporate these drugs into the treatment for HL and I believe these agents will greatly impact the treatment of HL in the future.
What are some of the treatment side effects?
The ABVD regimen is given once every two weeks. In general, it is reasonably well tolerated and the most common side effect is fatigue. Patients also commonly experience constipation, nausea (which is typically well controlled with anti-nausea medications), hair loss, and lowering of their blood counts. A less common, but serious side effect of ABVD, is pneumonitis. Pneumonitis is inflammation of the lungs that occurs in about 10% of patients exposed to bleomycin (the “B” in ABVD). This can cause shortness of breath and can rarely be fatal. For individuals who develop signs or symptoms of pulmonary toxicity while on treatment, we stop bleomycin and consider treatment with steroids.
Radiation therapy is often included in the front-line treatment for early stage HL. It was previously associated with significant toxicity, however it has become much safer throughout the years because the doses administered are lower, the fields used by radiation oncologists are smaller, and the technology has greatly improved. There is still concern though, particularly for women under 30 years of age, of increased risk for breast cancer for patients who receive radiation to the chest. There is also potential risk for acceleration of heart disease as well as for inflammation in the lungs for patients who receive radiation that extends into the lungs.
Brentuximab vedotin is currently available for patients with HL who have had their disease come back after at least two lines of therapy. It is an outpatient treatment that is generally well tolerated. The most common side effect is neuropathy, or numbness and tingling in the fingers and toes, which occurs in about half of the patients who receive the drug.
The anti-PD-1 agents, which are currently only available for HL patients through clinical trials, are also typically associated with only mild side effects. Current studies evaluating these agents exclude patients with histories of autoimmune diseases due to concern that these drugs can potentially aggravate these conditions. These agents can occasionly cause pneumonitis, colitis (inflammation in the bowel), or thyroid abnormalities as well.
How do you recommend patients combat these side effects?
It is helpful to eat a varied diet, drink a lot of fluids, get plenty of rest, and maintain some type of exercise program. Generally, I tell patients they need to listen to their bodies. If they are tired from exercising or tired in general, their body is telling them they need to take time to rest.
The most important thing for patients to do is communicate with their doctors. It is important for patients to discuss even mild symptoms with their doctor because what a patient perceives as mild might be an indication of something more serious.
Are there any treatments that can help minimize side effects?
We are fortunate to live in an era with excellent anti-nausea medications available and therefore significant nausea and vomiting is rarely seen with these chemotherapy regimens. Due to the risk of constipation with these regimens, many patients benefit from taking routine medications to combat constipation.
How did you become involved with the Lymphoma Research Foundation and why would you recommend patients become involved with the organization?
I presented at one of the Lymphoma Research Foundation's (LRF) patient education workshops and it was a really great experience. It was nice to meet patients and their families. The patients learned a lot about lymphoma and potential clinical trials that they possibly might not have heard about elsewhere. Because of that experience with LRF and the many additional patient services they provide, including the Lymphoma Support Network, Ask the Doctor programs and disease-specific websites and fact sheets, I certainly recommend that patients become involved with the Foundation and utilize their resources.