The Farber Center: For Radiation Oncology

• It is a type of lymphoma, a cancer that starts in cells called lymphocytes, which are part of the body's immune system.
• Because lymphoid tissue is found in many parts of the body, Hodgkin lymphoma can start almost anywhere.
• It often starts in lymph nodes in the upper part of the body. The most common sites are in the chest, in the neck, or under the arms.
• Hodgkin lymphoma most often spreads through the lymphatics to nearby lymph nodes in a stepwise fashion from lymph node to lymph node.
• Types of Hodgkin lymphoma: There are different types classified by how they look under the microscope. The 2 main types are Classic Hodgkin lymphoma (which has several subtypes) and nodular lymphocyte predominance Hodgkin lymphoma.
• Classic Hodgkin lymphoma accounts for about 95% of all cases. It has 4 subtypes, all of which have classic-appearing Reed-Sternberg cells. These cells are named after the 2 doctors who first described them. These cells are usually an abnormal type of B lymphocyte. Under a microscope, Reed-Sternberg cells are much larger than normal lymphocytes and also look different from the cells of non-Hodgkin lymphomas and other cancers. In Hodgkin lymphoma, the enlarged lymph nodes usually have a small number of Reed-Sternberg cells and a large number of normal immune cells. It is mainly these other immune cells that account for the bulkiness of the enlarged lymph nodes.
• Nodular sclerosis Hodgkin lymphoma: This is the most common type accounting for about 60% to 80% of cases. It occurs mainly in younger people, about equally in men and women. It tends to start in lymph nodes in the neck or chest.
• Mixed cellularity Hodgkin lymphoma: This is the second most common type (15% to 30%) and is seen mostly in older adults. It can start in any lymph node but most often occurs in the upper half of the body.
• Lymphocyte-rich Hodgkin lymphoma: This subtype accounts for about 5% of all cases. It usually occurs in the upper half of the body and is rarely found in more than a few lymph nodes.
• Lymphocyte-depleted Hodgkin lymphoma: This is the least common form making up only about 1% of cases. It is seen mainly in older people. The disease is more likely to be advanced when first found, involving lymph nodes in the abdomen as well as the spleen, liver, and bone marrow.
• Nodular lymphocyte predominant Hodgkin lymphoma accounts for about 5% of cases. It can occur at any age, and is more common in men than in women. This type usually involves lymph nodes in the neck and under the arm.
• All types of Hodgkin disease are malignant (cancerous) because as they grow they may compress, invade, and destroy normal tissue and spread to other tissues. There is no benign (non-cancerous) form of Hodgkin lymphoma.
• In general, Hodgkin lymphoma is most common in early adulthood (15-40y) and in late adulthood (after age 55y)
• It is most common in the US, Canada and northern Europe and least common in Asia.

• Depend on area of the body that is involved.
• It may not cause symptoms until it grows large.
• General symptoms can include:
• unexplained weight loss
• fever
• drenching night sweats (enough to soak clothing)

Risk Factors

• Family history: especially that of an identical twin having Hodgkin Lymphoma.
• Higher socioeconomic status.
• History of the EBV: causing virus mononucleosis (aka MONO)

Incisional or excisional biopsy: A biopsy in which only a sample of the suspicious tissue is cut into (incised) and removed for purposes of diagnosis. A incisional biopsy is in contrast to an excisional biopsy in which an entire lesion, usually a tumor, is removed.

History and physical exam including performance status and presence or absence of B-symptoms.

Laboratory studies Blood chemistry tests including protein levels, liver function tests, kidney function tests, and uric acid level.

Chest x-ray scans of the chest, abdomen, and pelvis.

Bone marrow biopsy for certain stages. A bone marrow biopsy is the removal of soft tissue from inside bone. Bone marrow grows inside some of the larger bones in the body. It produces platelets and red and white blood cells. The bone marrow biopsy may be done in the health care provider's office or in a hospital. The sample is usually taken from the hip bone. The skin is cleansed, and a local anesthetic is injected to numb the skin.
The biopsy needle is then inserted into the bone. The center of the needle is removed and the needle is moved deeper into the bone. This creates a tiny sample, or core, of bone marrow within the needle. The needle is then removed, along with the bone marrow sample. Pressure is applied to the biopsy site to stop bleeding, and a bandage is applied.
An aspirate may also be performed, usually before the biopsy is taken. After the skin is numbed, the aspirate needle is inserted into the bone, and a syringe is used to withdraw the liquid bone marrow. If this is done, the needle will be removed and either repositioned, or another needle may be used for the biopsy.

CT scan (computed tomography: This test uses a special x-ray machine that takes pictures from many angles. Before any pictures are taken, you may be asked to drink 1 to 2 pints of a liquid called oral contrast. This helps outline the intestine so that certain areas are not mistaken for tumors. You may also receive an IV (intravenous) line through which a different kind of contrast dye (IV contrast) is injected. This helps better outline structures in your body. A computer then combines these pictures into images of slices of the part of your body being studied. The CT scan can help show where stomach cancer is and where it has spread. CT scans can also be used to guide a biopsy needle into a place that might have cancer. The patient stays on the CT scanning table while a doctor moves a biopsy needle through the skin toward the tumor. A small piece of the tumor is removed and looked at under a microscope.

PET/CT may be helpful A PET scan measures important body functions, such as blood flow, oxygen use, and sugar (glucose) metabolism, to help doctors evaluate how well organs and tissues are functioning.
CT imaging uses special x-ray equipment, and in some cases a contrast material, to produce multiple images or pictures of the inside of the body. These images can then be interpreted by a radiologist on a computer monitor as printed images. CT imaging provides excellent anatomic information.
Today, most PET scans are performed on instruments that are combined PET and CT scanners. The combined PET/CT scans provide images that pinpoint the location of abnormal metabolic activity within the body. The combined scans have been shown to provide more accurate diagnoses than the two scans performed separately.

Other tests may be ordered depending on the location of the lymphoma.

The staging system most often used to describe the extent of non-Hodgkin lymphoma in adults is called the Ann Arbor staging system. The stages are described by Roman numerals I through IV (1-4). Lymphomas that affect organs outside of the lymph system (extranodal organs) have E added to their stage (for example, stage IIE), while those affecting the spleen have an S added.

Stage I
If either of the following is present it means the disease is stage I:
The lymphoma is in a lymph node or nodes in only 1 region, such as the neck, groin, underarm, and so on. The cancer is found only in 1 area of a single organ outside of the lymph system (IE).

Stage II
If either of the following is present it means the disease is stage II:
The lymphoma is in 2 or more groups of lymph nodes on the same side of (above or below) the diaphragm (the muscle that separates the chest and abdomen). For example, this might include nodes in the underarm and neck area but not the combination of underarm and groin nodes.
The lymphoma extends locally from a single group of lymph node(s) into a nearby organ (IIE). It may also affect other groups of lymph nodes on the same side of the diaphragm.

Stage III
If either of the following is present it means the disease is stage III:
The lymphoma is found in lymph node areas on both sides of (above and below) the diaphragm.
The cancer may also have spread into an area or organ next to the lymph nodes (IIIE), into the spleen (IIIS), or both (IIISE).

Stage IV
If either of the following is present it means the disease is stage IV:
The lymphoma has spread outside of the lymph system into an organ that is not right next to an involved node.
The lymphoma has spread to the bone marrow, liver, brain or spinal cord, or the pleura (thin lining of the lungs).
Along with the Roman numeral, each stage is also assigned an A or B. The letter B is added if any of the following "B symptoms" are present:

unexplained weight loss (more than 10% of weight)

soaking night sweats

unexplained fever of at least 101.5°F

These symptoms usually mean the disease is more advanced. If none of these B symptoms is present, the letter A is added to the stage.

Treatment, in general, depends on the stage, if it is considered “bulky”, and if there are B-symptoms.

SURGERY
is often used to biopsy and diagnose lymphoma. It rarely is used in the actual treatment.

BONE MARROW TRANSPLANT
A Bone marrow or peripheral blood stem cell transplant may also be part of the treatment depending how aggressive the Hodgkin lymphoma is.

CHEMOTHERAPY
The mainstay of treatment is chemotherapy and radiation therapy.
Occasionally, radiation therapy may be used by itself.
The most common chemotherapy regimens for the treatment of Classic Hodgkin Lymphoma include:

ABVD (Doxorubicin, Bleomycin, Vinblastine and Dacarbazine)

Stanford V (Doxorubicin, vinblastine, mechlorethamine, etoposide, vincristine, bleomycin and prednisone)

BEACOPP (Bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine and prednisone)

The most common chemotherapy regimens for the treatment of Nodular lymphocyte-predominant Hodgkin Lymphoma include:

ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) ± rituximab

CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) ± rituximab

CVP (cyclophosphamide, vincristine, prednisone) ± rituximab

EPOCH (cyclophosphamide, doxorubicin, etoposide, vincristine, prednisone) ± rituximab

Single agent rituximab