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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 good young gentleman. He was awarded a basketball scholarship to a state college, where he became the league’s leading scorer during his junior year. When his mother came to visit for a game late in the season, she noticed that Brian’s neck appeared much larger on one side than the other. The next day, she convinced him to see the college doctor who felt multiple enlarged lymph nodes in his right neck extending from the corner of his jaw down to just above his collarbone. The doctor ordered a CT scan of the neck and chest, which confirmed many abnormal lymph nodes in 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 wish biopsy revealed Hodgkin’s lymphoma.

Following 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 multiple lymph nodes. Brian was referred to a medical oncologist who recommended treatment with ABVD chemotherapy. He received 4 cycles, which he tolerated very well, with only moderate fatigue. A follow-up PET/CT scan revealed no residual abnormal activity. He was seen by a radiation oncologist who recommended low dose fractionated field radiation therapy (IFRT) after chemotherapy that was delivered over 3 weeks. Other than a mild sore throat, Brian tolerated 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 lymphoma (HL) is much less common than non-Hodgkin lymphoma (NHL), although it can be diagnosed in children as well as the elderly. If the disease is localized, then the 5-year survival rate is more than 90%. Even patients with more advanced HL have a 5-year survival rate of 75-80%

RISKS & CAUSES

People who have a history of a first-degree relative diagnosed with HL have a significantly increased risk of developing the disease themselves. 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 appears to be a correlation with patients of low socioeconomic status as well.

SIGNS & SYMPTOMS

The vast majority of patients with HL will come to the doctor with the complaint of an enlarged lymph node or multiple lymph nodes that will not go away. Most often the lump will arise in the neck, but can also be felt in other common lymph node areas including the armpit (under the arm) and the groin. Doctors should also ask questions about unexplained weight loss, fevers, or drenching night sweats, the “B symptoms,” which are classic for lymphoma. Although they are only present in a subset of patients, B symptoms tend to be predictive of more advanced disease. Rarely, patients may present with diffuse itching or redness of the skin when drinking alcohol as an initial 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 multiple subtypes of HL including: nodular sclerosing, mixed cellularity, lymphocyte rich and lymphocyte depleted. Yet another subtype, called nodular lymphocyte-predominant HL, appears to be biologically different from the others, but also has an extremely high cure rate.

Staging

A 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 latter two of which have been shown to be predictive of more advanced disease when elevated. As with NHL, the Ann Arbor staging system is used. This system is based on the number and locations 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 neck, chest, abdomen and pelvis. Wherever available, PET/CT is extremely useful for staging, radiation therapy (RT) planning, and assessment of response to therapy. Bone marrow biopsies are indicated for patients with advanced disease including those presenting with B symptoms. Other factors that may negatively affect outcome include male sex, age older than 45, 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. Regarding HL, four drugs are used most commonly 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 last few decades with ABVD has shown consistently excellent results.

Patients with early stage HL generally receive 2-6 cycles of ABVD. Restaging imaging is obtained after 2-4 cycles to guide further treatment. PET recovery is predictive of outcome. The combination of ABVD followed by fractionated field radiation therapy (IFRT) provides an excellent chance for a cure.

Common acute side effects of ABVD include fatigue, nausea (usually well controlled with medication), mild anorexia, decreased blood count, 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.

Because low doses and fairly small treatment areas are currently used for IFRT, side effects are much less than decades ago when the 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 pneumonia (inflammation of the lung) characterized by low-grade fever, dry cough and shortness of breath with exertion, which occurs classically 1-3 months after RT. The 5-10% of patients who develop pneumonia usually have resolution of their symptoms within 3-4 weeks of starting steroids. In the long term, despite the low doses and small RT fields, 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 who require RT to the breast should begin annual mammography (and usually breast MR) screening within 7-10 years of treatment or at age 40, whichever comes first.

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

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