Hodgkin’s Lymphoma

Hodgkin’s lymphoma (aka Hodgkin’s disease) is a malignancy of cells which, when functioning normally, participate in your body’s immune system. The cells typically circulate and are found in greatest number in your lymph nodes, which are found throughout the body.

Hodgkin’s lymphoma (HL) is bimodal in age distribution, meaning that it affects children and adolescents as well as adults. Individuals with a first-degree relative with HL have a 5-fold increase in eventually getting HL themselves.

The abnormal cells have a distinct look under a microscope, and the hallmark cell is called a Reed-Sternberg cell. It has two large nuclei. Special studies looking at structures on the surface of the tumor cells help differentiate HL with non-Hodgkin’s lymphoma (NHL); in “classic” HL we see CD15 and CD30 positivity.

Patients with HL present in various ways. Most will present with enlarged cervical (neck) nodes, while half will present with lymph nodes in the mediastinum (central chest above heart). Approximately 1/3 will have what are called “B symptoms” which are defined at weight loss of >10% in 6 months, fevers of >38 degrees Celcius, or drenching night sweats.

The various types of “classic” HL are nodular sclerosing (NS), mixed cellularity (MC), lymphocyte predominant (LP), and lymphocyte depleted (LD). Another, less aggressive subtype is called nodular lymphocyte predominant hodgkin’s lymphoma (NLPHL).

Initial workup for HL includes an excisional biopsy, where a large the tumor is actually removed rather than just biopsied by a small needle. This is done to assist in making an accurate diagnosis. Additional studies may include a bone marrow biopsy, extensive bloodwork, CT and PET scans.

Patients are staged using the Ann Arbor staging system, which essentially breaks patients down to “early” or “advanced”. Early stages are those with disease limited to one side of the diaphragm, while advanced have tumors on both sides of the diaphragm or within the bone marrow. In addition, patients will be assessed as to whether or not they have “favorable” factors, which essentially means that they have non-bulky (<10 cm or less than 1/3 intrathoracic diameter) tumor, less than or equal to 3 involved sites, no B-symptoms, and an ESR of less than 50.

Prognosis in general for patients who undergo therapy is excellent, even for more advanced stages. Early favorable tumors have a 95% freedom from failure, while those with multiple risk factors have freedom from failure ranging from 50-70%.

Treatment generally consists of a combination of chemotherapy and radiation, with 4 cycles of chemo followed by a small field of radiation in patients with stage I disease and an additional couple of cycles of chemo with the same radiation in patients with B symptoms and other unfavorable characteristics. Stage III and IV patients are also treated with chemotherapy up front, but are re-staged after 4 cycles and then go on to receive additional chemotherapy followed by radiation. Tumors that don’t respond to the above approach will need to be evaluated for high dose chemotherapy and stem cell rescue. Those tumors that initially regress but then recur can be treated in a variety of ways depending on how the tumor was treated the first time, but in general it includes some combination of chemotherapy and radiation.

Side effects of the treatment are similar from a chemotherapy standpoint regardless of the site of the tumor, and are best discussed with your medical oncologist. The radiation side effects will depend on the site that it being treated. Unlike chemotherapy, radiation therapy is a local/regional therapy, which means that if your radiation oncologist is treating, say, your chest and axilla, you will not lose facial or scalp hair and will not likely have problems with nausea or diarrhea.

Treatment guidelines have been substantiated by multiple studies both here in the United States and also Europe, primarily in Germany. We therefore have excellent evidence that the treatment protocols discussed above result in excellent oncologic outcomes.

Follow up will usually be done through your medical oncologist every 3 months or so and will include surveillance imaging and bloodwork, along with comprehensive physical exam. Data shows that patients undergoing therapy for HL are at long-term risk for leukemia and solid tumors above the general public baseline. In addition, it is critical for women treated at a young age to start surveillance mammography 5-8 years after completion of radiotherapy to catch any radiation-induced breast cancers at an early stage.