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Hodgkin 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 with 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 him for a game at the end of the season, she noticed that Brian’s neck seemed much larger on one side than the other. The next day, she convinced him to see the university doctor who felt multiple enlarged lymph nodes on the right side of his neck extending from the angle of his jaw to just above his clavicle. The doctor ordered a neck and chest CT scan which confirmed multiple abnormal lymph nodes on the right side of the neck and in the center of the chest. Brian was referred to a general surgeon who removed one of the largest lymph nodes in his neck during outpatient surgery. This excisional biopsy revealed Hodgkin lymphoma.

Following his diagnosis, Brian was sent for a bone marrow biopsy which came back negative. He also underwent a PET/CT scan which confirmed abnormal activity on the right side of his neck and chest in multiple 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 moderate fatigue. A restaging PET/CT scan revealed no residual abnormal activity. He was seen by a radiation oncologist who recommended low-dose involved-field radiotherapy (IFRT) after chemotherapy that was given for 3 weeks. Aside from a slight sore throat, Brian tolerated RT quite well. He was seen every 3 to 6 months by his medical and radiation oncologist for alternate follow-up visits. Brian has been disease free for 7 years.

THE ESSENTIAL

Hodgkin lymphoma (HL) is much less common than non-Hodgkin lymphoma (NHL), although it can be diagnosed in both children and the elderly. If the disease is localized, 5-year survival is greater than 90%. Even the most advanced HL patients 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 have a significantly higher risk of developing the disease. Furthermore, patients who have an Epstein-Barr virus (EBV) infection early in life appear to be at increased risk of HL later in life. There also appears to be a correlation with patients of low socioeconomic status.

SIGNS AND SYMPTOMS

The vast majority of HL patients will present to the doctor with the complaint of an enlarged lymph node or multiple lymph nodes that do not go away. Most often, the node will originate in the neck, but it can also be felt in other common lymph node areas, such as the armpit (under the arm) and groin. Doctors should also ask about unexplained weight loss, fever, or night sweats, the “B symptoms” that are classic lymphoma. Although present in only a subset of patients, B symptoms tend to predict more advanced disease. Rarely, diffuse itching or redness of the skin may occur when patients drink alcohol as an early sign of LH.

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 multiple subtypes of HL including: nodular sclerosing, mixed cellularity, lymphocyte-rich, and lymphocyte-depleted. Another subtype, called nodular lymphocyte-predominant HL, appears to be biologically different from the others, but it also has an extremely high cure rate.

STAGING

Standard laboratory evaluation should include complete blood count, serum chemistry including kidney and liver function, blood lactate dehydrogenase (LDH) levels, and erythrocyte sedimentation rate (ESR), the latter two of which have been shown to predict more advanced disease when elevated . As with the 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 B symptoms. Imaging should include CT of involved areas, including the neck, chest, abdomen, and pelvis. Whenever available, PET/CT is extremely useful for staging, radiation therapy (RT) planning, and assessment of response to treatment. Bone marrow biopsies are indicated for patients with advanced disease, including those presenting with B symptoms. Other factors that may have a negative impact on the result include male gender, age greater than 45 years, low serum hemoglobin, high white blood cell count. , low albumin and stage IV disease.

TREATMENT

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

Early-stage HL patients generally receive 2 to 6 cycles of ABVD. Restaging images are obtained after 2 to 4 cycles to guide subsequent treatment. PET restaging is predictive of outcome. The combination of ABVD followed by involved field radiotherapy (IFRT) offers an excellent chance of cure.

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

Since low doses and fairly small treatment areas are currently used for IFRT, the 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 (lung inflammation) characterized by low-grade fever, dry cough, and shortness of breath on exertion, which classically occurs 1 to 3 months after RT. 5-10% of patients who develop lung inflammation usually have a resolution of their symptoms within 3-4 weeks of starting steroids. In the long term, despite the low doses and small RT fields, there is still a small risk of developing radiation-induced cancer years after treatment. Patients should be encouraged to stop smoking prior to RT. Adolescents and young women who require chest RT should start an annual mammogram (and usually a breast MRI) within 7 to 10 years of treatment or age 40, whichever comes first.

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