Hodgkin Lymphoma: Stages and Treatment

Published on September 11, 2023 .

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Hodgkin Lymphoma cells under microscope — Image by Nemes Laszlo via Shutterstock

Hodgkin lymphoma (HL) is a cancer of the lymphatic system of the body. It makes up 10% of lymphomas diagnosed each year. HL is staged and then treated accordingly with chemotherapy, radiation, immunotherapy, or transplant. With initial treatment, HL is highly responsive and has an 80–95% cure rate.

Hodgkin lymphoma is a cancer of the lymphatic system. The incidence is 2–3 out of every 100,000 people.
Hodgkin lymphoma occurs most often in young adults ages 15–40 years and peaks again at age 55+.
The most common sites of disease are the lymph nodes, spleen, liver, and bone marrow.
The treatment for Hodgkin lymphoma may consist of chemotherapy, radiation, immunotherapy, or stem cell transplant, and the treatment is intended to be curative.

Types of Hodgkin lymphoma

The lymphomas overall divide into Hodgkin and non-Hodgkin lymphomas. The two main types of Hodgkin lymphoma are classical Hodgkin lymphoma (CHL) and nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL). Risk factors include male sex, age, a family history of Hodgkin lymphoma, immunosuppressive conditions, like human immunodeficiency virus (HIV), or a prior history of the Epstein-Barr virus (EBV).

Symptoms of Hodgkin lymphoma

Hodgkin lymphoma (HL) has a variety of symptoms at presentation. Symptoms include:

Unintentional weight loss
Soaking night sweats
Fatigue
Lack of appetite or feeling full fast with eating

Cough or shortness of breath
Swelling or abdominal pain
Fever or chills
Painful lymph nodes with drinking alcohol
Diffuse itching

In 30% of patients, fever, unintentional weight loss, and night sweats, also known as B-symptoms, are present. These symptoms often lead to an evaluation by a healthcare provider who can start the process of diagnosing HL.

How is Hodgkin lymphoma diagnosed?

Your healthcare provider will order tests to evaluate you for Hodgkin lymphoma. These typically include blood work, including an erythrocyte sedimentation rate (ESR) and complete blood count (CBC), computerized tomography (CT) scans, and a positron emission tomography (PET) scan.

Once there is an area or lymph node found that can be biopsied, a pathologist reviews the biopsy sample to confirm the diagnosis. A bone marrow biopsy assesses if the HL has spread to the bone marrow. HL staging occurs after the test results are available.

Staging Hodgkin lymphoma

There are four stages of HL, per the Lugano classification.

Stage	Symptoms
Stage I	HL is in one lymph node or one lymph organ. If HL is outside the lymph system in one location, it is Stage IE.
Stage II	HL is in two lymph nodes on the same side of the diaphragm (above or below). If HL is in one lymph node and extends into an adjacent organ, it is Stage IIE.
Stage III	HL is in lymph nodes above and below the diaphragm. HL is in lymph nodes (above or below the diaphragm) AND in the spleen.
Stage IV	HL is in at least one organ outside the lymphatic system such as the bone marrow, lungs, bone, or liver.

Additional staging classifications include:

In any stage, if B-symptoms are absent, an “A” is added to the stage. Example: Stage IIIA.
In any stage if B-symptoms are present a “B” is added to the stage. Example: Stage IIIB. The presence of B-symptoms requires more intense treatment.
If the spleen is involved, an “S” is added to the stage. Example: Stage IIIS.
Tumors greater than 4 inches (10 cm) in size or that comprise more than 1/3 of the space in the chest, it is considered “bulky.” For bulky disease, more intense treatment is needed.

Treatment of CHL

Treatment of classical Hodgkin lymphoma is based on the patient's health status, age, and stage. For stage IA or IIA that is considered to be favorable (no bulky disease, no B-symptoms, non-elevated ESR, no cancer outside the lymph nodes, and with disease location limited to 3 lymph node areas), 2–4 cycles of chemotherapy followed by radiation is recommended. Another option is 3–6 cycles of chemotherapy alone.

Unfavorable stage IA or IIA HL has >1 of the following: bulky disease, the presence of B-symptoms, an elevated ESR, extranodal (outside the lymph node) involvement, or HL in three or more areas of lymph nodes. Unfavorable disease requires more intensive treatment for 4–6 cycles of chemotherapy. More chemotherapy (same or different kind) may be indicated if imaging done during treatment shows persistent or worsening disease. This is followed by radiation to any bulky sites of disease.

Stage III and IV Hodgkin lymphoma are treated with chemotherapy for longer courses, often 6–8 cycles. If imaging done during treatment shows persistent or worsening disease, more chemotherapy is required. Radiation to any large sites of disease may be recommended. An added antibody-drug conjugate infusion could be suggested with chemotherapy or continued afterward.

Treatment of NLPHL

Early-stage nodular lymphocyte-predominant HL is treated with radiation alone if there are no B-symptoms or bulky disease present. If B-symptoms or bulky disease is present, more intensive chemotherapy is given, with or without immunotherapy, followed by radiation.

For stage III or IV NLP-HL, chemotherapy and immunotherapy, with or without radiation, are suggested.

Recurrent or refractory HL

If chemotherapy and radiation therapy do not work, it may be necessary to try different high-dose chemotherapy or added radiation. This may be followed by a stem cell transplant.

If HL comes back later, depending on the initial treatment, chemotherapy, radiation, immunotherapy, or a stem cell transplant are possible options.

Pregnant patients and children with HL

The treatment of HL in children and in pregnant patients is not covered in this article. The treatments are different, and variable based on the type of HL and the stage.

Prognosis of HL

Hodgkin lymphoma overall has excellent cure rates. In stages I to III, the 5-year relative survival (OS) is, on average, 85–95%. Persons with stage IV disease have a 5-year relative survival of about 80%. These percentages may be even better in the last few years, given that there have been advances in treatment options that are unaccounted for in the data. In summary, HL is a treatable and highly curable cancer.