When To Worry About A Fever In 1 Year Old Hodgkin’s Lymphoma and Brian’s Story: A Highly Curable Cancer

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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 shape and had no 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 State College, where he became the league’s leading scorer during his junior year. When his mother came to watch a game late in the season, she noticed that Brian’s neck looked much bigger on one side than the other. The next day, he convinced her to see the university doctor, who felt several enlarged lymph nodes in her right neck, extending from the corner of her jaw down to above her 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 and in the center of the chest. Brian was referred to a general surgeon who removed one of the larger lymph nodes in his neck on an outpatient basis. This surgical biopsy revealed Hodgkin’s lymphoma.

After the diagnosis, Brian was sent for a bone marrow biopsy, which was negative. He also underwent a PET/CT scan which confirmed abnormal activity in several lymph nodes in the right neck and chest. Brian was referred to an 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 abnormal activity. She was seen by a radiation oncologist who recommended low-dose field radiation therapy (IFRT) after chemotherapy, which was given over three weeks. Except for a mild sore throat, Brian tolerated RT quite well. He was seen by his medical and radiation oncologist every 3 to 6 months for alternating 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 both children and the elderly. If the disease is localized, the 5-year survival rate is over 90%. Even in patients with advanced HL, the 5-year survival rate is 75-80%.

RISKS AND CAUSES

People who have had a first-degree relative diagnosed with HL have a significantly higher risk of developing the disease themselves. In addition, patients with Epstein-Barr virus (EBV) infection early in life appear to have an increased risk of HL later in life. There seems to be a correlation also with patients of low socio-economic status.

SIGNS AND SYMPTOMS

Most patients with HL come to the doctor complaining of an enlarged lymph node or multiple lymph nodes that do not go away. Most often, a node occurs in the neck, but it can also be felt in other common lymph node areas, including the armpit (under the arm) and groin. Doctors should also ask questions about unexplained weight loss, fever, or night sweats, the “B symptoms” that are classic in the treatment of lymphoma. Although present in only a minority of patients, B symptoms tend to predict advanced disease. Rarely, patients may experience patchy itching or reddening of the skin when drinking alcohol as the first sign of HL.

DIAGNOSIS

Like NHL, the preferred biopsy method for HL is complete surgical removal (excisional biopsy) of the enlarged lymph node whenever possible and safe. There are several subtypes of HL, including: nodular sclerosing, mixed cell, lymphocytic, and lymphocytic. Yet another subtype, called nodular lymphocyte-predominant HL, appears to be biologically different from the others, but also has a very high cure rate.

SET

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

CARE

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 abbreviation for the combination is ABVD. The treatment of tens of thousands of ABVD patients over the past decades has shown consistently excellent results.

Patients with early-stage HL usually receive 2 to 6 cycles of ABVD. Repeat imaging is obtained after 2–4 cycles to guide further treatment. PET reprocessing predicts outcome. The combination of ABVD followed by in-field radiation therapy (IFRT) gives an excellent chance of cure.

Common acute side effects of ABVD include fatigue, nausea (usually well controlled with medication), mild anorexia, decreased blood counts, and hair loss. Rare but serious late post-treatment side effects include adriamycin-induced heart damage, bleomycin-induced lung damage, and vinblastine-induced nerve damage. Chemotherapy increases patients’ risk of future cancers, most commonly leukemia or NHL.

Because IFRT currently uses low doses and fairly small treatment areas, there are far fewer side effects than decades ago when doses were higher and treatment areas were larger. Common acute side effects of IFRT include mild fatigue, possible partial hair loss (hair loss), and sore throat or difficulty swallowing depending on the area treated. There is a risk of pneumonia (pneumonia), characterized by low-grade fever, dry cough, and dyspnea on exertion, which traditionally occurs 1-3 months after RT. In the 5–10% of patients who develop pneumonia, symptoms usually resolve within 3–4 weeks of starting steroids. In the long term, despite the small doses and small RT fields, there is a small risk of developing radiation-induced cancer years after treatment. Patients should be encouraged to stop smoking before RT treatment. Teenage girls and young women who require chest RT should begin annual screening mammography (and usually breast MRI) within 7 to 10 years after treatment or at age 40, whichever comes first.

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

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