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Hodgkin’s Lymphoma and Brian’s Story: A Highly Curable Cancer

BRIAN’S STORY

Brian was a 20-year-old college basketball star who was in excellent condition and had no history of health problems. He grew up in a tough neighborhood with a single mother who raised him to be a fine young gentleman. He was awarded a basketball scholarship to the state university, where he became the league’s leading scorer during his junior year. When his mother came to visit for a game at the end of the season, she noticed that Brian’s neck looked much bigger on one side than the other. The next day, he convinced to see the university doctor who felt multiple enlarged lymph nodes in the right neck extending from the angle of the jaw to just above the collarbone. The doctor ordered a CT scan of the neck and chest which confirmed several abnormal lymph nodes on the right side of the neck as well as the center of the chest. Brian was referred to a general surgeon who removed one of the larger lymph nodes in his neck during outpatient surgery. This excisional biopsy revealed Hodgkin lymphoma.

After his diagnosis, Brian was sent for a bone marrow biopsy which was negative. He also underwent a PET/CT scan which confirmed abnormal activity in the right neck and chest in several lymph nodes. Brian was referred to a medical oncologist who recommended treatment with ABVD chemotherapy. He received 4 cycles that he tolerated very well, with only moderate fatigue. A staging PET/CT scan revealed no residual abnormal activity. He was seen by a radiation oncologist who recommended a low dose involved field radiotherapy (IFRT) after chemotherapy which was delivered in 3 weeks. Other than a mild sore throat, Brian is tolerating RT quite well. He was seen every 3-6 months by his medical and radiation oncologist for alternate follow-up visits. Brian has been disease free for 7 years.

BASICS

Hodgkin’s lymphoma (HL) is much less common than non-Hodgkin’s lymphoma (NHL), although it can be diagnosed in children as well as in the elderly. If the disease is localized, the 5-year survival is more than 90%. Even patients with more advanced HL have a 5-year survival rate of 75-80%

RISKS AND CAUSES

People who have a history of a first-degree relative diagnosed with HL are at a significantly increased risk of developing the disease. In addition, patients who have Epstein-Barr virus (EBV) infection early in life appear to have an increased risk of HL later in life. There is also a correlation with patients of low socioeconomic status.

SIGNS & SYMPTOMS

Most patients with HL come to the doctor with the complaint of an enlarged lymph node or several lymph nodes that do not go away. Most often, the node will arise in the neck, but it can also be felt in other common lymphatic areas including the axilla (under the arm) and the groin. Doctors also ask questions about unexplained weight loss, fever or night sweats, the “B symptoms” that are classic for lymphoma. Although present in only a subset of patients, B symptoms tend to predict for more advanced disease. Rarely, patients may experience diffuse itching or reddening of the skin when drinking alcohol as an early sign of HL.

DIAGNOSIS

Like NHL, the preferred method of biopsy for HL is complete surgical removal of an enlarged lymph node (excisional biopsy) whenever feasible and safe. There are several subtypes of HL including: nodular sclerosing, mixed cellularity, rich in lymphocytes and depleted lymphocytes. However, another subtype, called nodular lymphocyte predominant HL, appears to be biologically different from the others, but also has an extremely high cure rate.

MISE EN SCENE

Standard laboratory evaluation should include a complete blood count, serum chemistries including renal and liver function, blood levels of lactate dehydrogenase (LDH) and erythrocyte sedimentation rate (ESR), the the latter two have been shown to predict more advanced disease when elevated. As with NHL, the Ann Arbor staging system is used. This system is based on the number and sites of involvement in lymph nodes and other organs, as well as the presence or absence of symptoms B. The image must include CT of the involved areas including the neck, chest, the abdomen and the pelvis. Wherever available, PET/CT is extremely useful for staging, planning radiotherapy (RT) and evaluating response to treatment. Bone marrow biopsies are indicated for patients with advanced disease including those with symptoms B. Other factors that can negatively impact the outcome include male sex, age over 45 years, low serum hemoglobin, white blood cell count, low albumin and stage IV disease.

TREATMENT

Like NHL, HL is treated with a combination of chemotherapy drugs. In the case of HL, four drugs are used most commonly in the United States: adriamycin, bleomycin, vinblastine and dacarbazine. The acronym for the combination is ABVD. The treatment of tens of thousands of patients over the past decades with ABVD has shown excellent results.

Patients with early stage HL usually receive 2-6 cycles of ABVD. The restaging image is obtained after 2-4 cycles to guide further treatment. PET scan is predictive of outcome. The combination of ABVD followed by radiation therapy of the involved field (IFRT) gives an excellent chance for a cure.

Common acute side effects of ABVD include fatigue, nausea (usually well controlled with medication), mild anorexia, low blood pressure, and hair loss. Uncommon but serious late side effects after treatment include heart damage from adriamycin, lung damage from bleomycin, and nerve damage from vinblastine. Chemotherapy puts patients at a slightly increased risk for developing future cancers, most commonly leukemia or NHL.

Since low doses and fairly small treatment areas are currently used for IFRT, side effects are much less than decades ago when doses were higher and treatment areas were larger. Common acute side effects of IFRT include mild fatigue, possible partial alopecia (hair loss), and sore throat or difficulty swallowing, depending on the area treated. There is a risk of pneumonitis (inflammation of the lung) characterized by a low-grade fever, dry cough and shortness of breath with exertion, which is classically found 1-3 months after RT. 5-10% of patients who develop pulmonary inflammation usually resolve their symptoms within 3-4 weeks after starting steroids. In the long term, despite the low doses and small fields of RT, there remains a small risk of developing radiation-induced cancer years after treatment. Patients should be encouraged to quit smoking before RT. Adolescents and young women requiring breast RT should begin annual mammography (and usually breast MRI) screening 7-10 years after treatment or at age 40, whichever comes first.

Advanced stage HL is most commonly treated with systemic chemotherapy alone, again mainly ABVD in the US Cure rates are around 70%. Patients can receive 6-8 cycles of ABVD, with PET/CT restaging performed after 4-6 cycles to assess response. The role of consolidative IFRT for these patients is controversial.

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