Adult Hodgkin Lymphoma: Staging and Treatment

What is staging for cancer?

Staging helps you learn where your cancer is, how much is in your body, and if it has spread. For Hodgkin Lymphoma (HL), which is a cancer of the lymph nodes and lymphatic tissue, a biopsy is done of the lymph nodes and the tissue is looked at under a microscope to make the diagnosis of Hodgkin lymphoma. There are two types of HL: classical and nodular lymphocyte-predominant (NLP). You may have other tests done to help determine the stage of your HL:

• Blood tests, like a complete blood count (CBC), liver function tests, kidney function tests, erythrocyte sedimentation rate (ESR, a marker of inflammation), and a pregnancy test in women of childbearing age.
• PET-CT scan (combines a CT scan of the body (a 3D x-ray) with PET scan).
• Bone marrow aspiration and biopsy
• Echocardiogram
• Pulmonary Function Test
• X-Ray
• MRI

How is Hodgkin lymphoma staged?

Your care team will stage your cancer. Staging looks at how much disease you have and if/where it has spread. Staging helps guide your treatment plan. The staging system for Hodgkin lymphoma is known as the Ann Arbor staging system (Cotswold modification). It has four different stages:

• Stage I: A single lymph node region is involved, or localized involvement of a single extralymphatic organ or site. Also known as early stage.
• Stage II: Involvement of 2 or more lymph node regions on the same side of the diaphragm or localized involvement of a single associated extralymphatic organ or site and its regional lymph nodes, with or without the involvement of other lymph node regions on the same side of the diaphragm. Also known as locally advanced disease.
• Stage III: Involvement of lymph node regions on both sides of the diaphragm, which may also be accompanied by localized involvement of associated extralymphatic organ or site, by involvement of the spleen, or by both. Also known as advanced disease.
• Stage IV: Disseminated (multifocal) involvement of one or more extralymphatic organs, with or without associated lymph node involvement, or extralymphatic organ involvement with distant (nonregional) nodal involvement. Also known as widespread disease.

Early-stage Hodgkin lymphoma (Stage I-II) is divided further into two groups, "favorable" and "unfavorable," to help predict which patients may benefit from more aggressive treatment. The criteria for unfavorable disease are as follows:

• Bulky mediastinal disease or bulky disease (tumor >10 cm in size).
• Extension outside of lymph nodes ("extranodal" disease).
• Involvement of three or more lymph node areas, or ESR > 50 mm. The erythrocyte sedimentation rate (ESR) is a laboratory test that measures the rate at which red blood cells sediment in a period of one hour.

Your cancer staging may also include letters:

• E: There is an extension of the disease affecting an organ outside of the lymph system.
• S: The disease involves the spleen.
• B: The patient has presented with "B" symptoms related to the disease including: loss of more than 10% of body weight over the past 6 months, fever of 100.4 F or higher, or drenching night sweats.
• A: The patient has had no "B" symptoms.
• X: The patient has bulky disease. Bulky disease describes tumors in the chest that are at least one-third as wide as the chest.

Some other terms used when describing Hodgkin lymphoma are "resistant" or "progressive" disease. These terms are used when treatment does not make the disease go away or the disease gets worse. "Recurrent" or "relapsed" disease are the terms used when the disease went away with treatment but has returned in either the same place or in another part of the body.

How is Hodgkin lymphoma treated?

Treatment for Hodgkin lymphoma depends on the stage of the disease, whether it is favorable or unfavorable, your current health, and your goals for treatment.

There are different types of treatments for NL:

• Chemotherapy.
• Targeted Therapy.
• Radiation.
• Stem Cell/Bone Marrow Transplant.
• Clinical Trials.

Chemotherapy

Chemotherapy is medications that attack fast-growing cells and can help treat HL. Chemotherapy is known as a "systemic" treatment, which means that it goes throughout the entire body. These medications may be given through a vein (IV, intravenously), by mouth, or directly into the spinal fluid. Chemotherapy is often used to treat Hodgkin lymphoma. Some chemotherapy regimens may be given before treatment with radiation. The most common chemotherapy regimens used are called ABVD, BEACOPP, and Stanford Five (V).

• ABVD: adriamycin, bleomycin, vinblastine, and dacarbazine.
• BEACOPP: bleomycin, etoposide, adriamycin, cyclophosphamide, vincristine, procarbazine, and prednisone.
• Stanford V: doxorubicin, vinblastine, mechlorethamine, vincristine, bleomycin, etoposide, and prednisone.

You may receive one of these regimens or a combination of other medications. Your provider can explain why they recommend one regimen over another.

Targeted Therapy

Targeted therapy is a type of medication that works by targeting specific genes or proteins that are helping the cancer to grow. The most common targeted therapy used in the treatment of Hodgkin lymphoma, especially in the treatment of NDL, is rituximab. Rituximab can be given by itself or in combination with chemotherapy and/or radiation. Brentuximab vedotin is often used for patients whose disease has returned after other treatment regimens. Other targeted therapies used in the treatment of relapsed or refractory Hodgkin lymphoma include everolimus, nivolumab, and pembrolizumab.

Radiation Therapy

Radiation therapy is the use of high-energy x-rays to kill cancer cells. Radiation therapy may also be done as a palliative treatment. This means it is done to help with symptoms, such as bleeding, pain, or problems caused by metastasis (especially to the brain and bones).

There are two main types of radiation therapy used to treat Hodgkin lymphoma: photon (traditional radiation) and proton therapy. Proton therapy is only available at certain centers. Many radiation oncologists now choose to treat just the lymph nodes that are involved and the surrounding areas where the tumor has spread (called involved site radiation therapy, ISRT). You should talk with your provider about which type of radiation therapy is right for you.

Stem Cell/Bone Marrow Transplant

Transplants are used for some patients who have Hodgkin lymphoma who have received chemotherapy and/or radiation therapy, but the HL is still present. Transplants can be done using a donor's bone marrow or stem cells (allogeneic) or a patient's own bone marrow or stem cells (autologous).

Clinical Trials

You may be offered a clinical trial as part of your treatment plan. To find out more about current clinical trials, visit the OncoLink Clinical Trials Matching Service.

Making Treatment Decisions

Your care team will make sure you are included in choosing your treatment plan. This can be overwhelming as you may be given a few options to choose from. It feels like an emergency, but you can often take a few weeks to meet with different providers and think about your options and what is best for you. This is a personal decision. Friends and family can help you talk through the options and the pros and cons of each, but they cannot decide for you. You need to be comfortable with your decision – this will help you move on to the next steps. If you ever have any questions or concerns, be sure to call your team.

You can learn more about Hodgkin Lymphoma at OncoLink.org.