Lymphoma is a cancer of lymphocytes, which are white blood cells of the immune system that are located in lymph nodes throughout the body. Lymphoma is divided into two main subtypes: Hodgkin lymphoma (HL) and non-Hodgkin Lymphoma (NHL). Non-Hodgkin lymphoma is a general term used for multiple other sub-types of lymphoma which are discussed elsewhere. Hodgkin lymphoma is a specific type of lymphoma whose diagnosis is made based on a characteristic appearance of the cells under the microscope. The classic malignant cell type seen in HL under the microscope is the Reed-Sternberg cell, which distinguishes this disease from NHL.
HL is uncommon, with an average incidence of 8,000 cases per year in the United States. It is most commonly seen in young patients, with an average age of 30, though some people will develop the disease later in life.
Symptoms of Hodgkin Lymphoma
Common symptoms associated with HL include non-painful, swollen lumps or masses (lymph nodes) in the neck, under the arms, in the abdomen, or in the groin. There are other areas of the body that contain lymph nodes, including around the heart and lungs, as well as deep in the abdomen and pelvis. These nodes can also be affected by HL, and symptoms such as chest pain, cough, shortness of breath, abdominal pain, or problems with the bowels may occur depending on the location of the swollen nodes. Some patients may also have fevers, night sweats, generalized skin itching, and/or weight loss. Doctors call these “B-symptoms”.
Risk Factors for Developing Hodgkin’s Disease
There is a very small increase in risk of developing HL in relatives of patients with HL, however this is not entirely clear. There is also a slightly increased risk in people with a history of infectious mononucleosis caused by the Epstein-Barr virus. Smoking may also increase the risk of developing HL.
How Hodgkin Lymphoma is Diagnosed
Occasionally patients will be diagnosed after being found to have an abnormal chest x-ray that was done for other reasons (yearly physical, prior to surgery, etc.). For patients who are seen by their primary care physicians with specific complaints, they are often sent for x-rays or computed tomography (CT) scans based on physical signs and symptoms. Patients are then sent to surgeons, who will biopsy one of the lymph nodes (either the ones that can be felt on physical exam or ones that are seen on CT scans). The biopsy is sent to the lab, where a pathologist will examine it under a microscope. HL has a characteristic appearance under the microscope, which is recognized and diagnosed by the pathologist. The cell that is most characteristic of HL is the Reed-Sternberg cell.
Once the diagnosis is made, patients will often be sent for further testing. This is called “staging”. Prior to starting treatment, patients will need blood tests, CT scans of the neck, chest, abdomen, and pelvis if they were not done initially, testing of heart function with either an echocardiogram or Multi Gated Acquisition Scan (MUGA) and sometimes a Positron emission tomography (PET) scan. A PET scan is similar to a CT scan and can be helpful in determining the extent of disease and it serves as a point of comparison to measure how well a patient is responding to treatment. In addition, all patients require an initial bone marrow biopsy to rule out bone marrow involvement with HL.
HL is divided into four stages based on the extent of lymph nodes involved with disease. The current staging system used is called the Ann-Arbor staging system. The system is as follows:
- Stage I – Involvement of one lymph node region (I), or a single non-lymphatic organ or site (Ie).
- Stage II – Involvement of two or more lymph node regions on the same side of the diaphragm (II) or of one lymph node region and an adjacent non-lymphatic organ or site (IIe).
- Stage III - Involvement of lymph node regions on both sides of the diaphragm, which may include the spleen (IIIs) and/or a non-lymphatic organ or site (IIIe, IIIes).
- Stage IV - Involvement of one or more non-lymphatic organs.
Each stage also includes a letter, A or B, to indicate whether fever, weight loss, or night sweats are present. "A" means these symptoms are not present; "B" means they are.
Treatment Options for Hodgkin Lymphoma at Tufts Medical Center
HL is treated with a combination of chemotherapy and sometimes radiation therapy, both of which are offered at Tufts. The most common treatment regimen used is called “ABVD” with stands for a combination of four chemotherapy drugs: Adriamycin, Bleomycin, Vinblastine, and Dacarbazine. These are IV infusions (given by vein) administered over the course of several hours, and are given in our infusion center on South 8. Admission to the hospital is not usually required for this treatment. Based on the extent of disease present, some patients require radiation therapy, which is also available.
Hodgkin lymphoma, particularly in those patients who are in Stage I or II, can be cured in some 80% of patients with this upfront treatment.
If a patient relapses or does not respond to initial therapy, another course of chemotherapy is recommended at the time of relapse. There are various combinations of chemotherapy drugs that can be used in this situation, and the choice of regimen varies depending on each patient’s medical history. If a patient responds well to a second course of chemotherapy and meets other specified medical criteria, autologous stem cell transplant (transplant using a patient’s own bone marrow stem cells) provides the best option for cure. If a patient relapses again after autologous stem cell transplant, further options include more chemotherapy and possibly an allogeneic bone marrow transplant (transplant of bone marrow cells from a matched relative or matched unrelated donor).
All of the above mentioned therapies are available and commonly performed at Tufts Medical Center's Cancer Center.