Hodgkin's lymphoma — formerly known as Hodgkin's disease — is a cancer of the lymphatic system, which is part of your immune system.
In Hodgkin's lymphoma, cells in the lymphatic system grow abnormally and may spread beyond the lymphatic system. As Hodgkin's lymphoma progresses, it compromises your body's ability to fight infection.
Most Hodgkin lymphomas are the classical type. The classical type is broken down into the following four sub-types:
- Nodular sclerosing Hodgkin lymphoma.
- Mixed cellularity Hodgkin lymphoma.
- Lymphocyte depletion Hodgkin lymphoma.
- Lymphocyte-rich classical Hodgkin lymphoma
Patients with Hodgkin's disease may present with the following symptoms:
- Lymph nodes: the most common symptom of Hodgkin's is the painless enlargement of one or more lymph nodes, or lymphadenopathy. The nodes may also feel rubbery and swollen when examined. The nodes of the neck and shoulders (cervical and supraclavicular) are most frequently involved (80–90% of the time, on average). The lymph nodes of the chest are often affected, and these may be noticed on a chest radiograph.
- Itchy skin
- Night sweats
- Unexplained weight loss
- Splenomegaly: enlargement of the spleen occurs in about 30% of people with Hodgkin's lymphoma. The enlargement, however, is seldom massive and the size of the spleen may fluctuate during the course of treatment.
- Hepatomegaly: enlargement of the liver, due to liver involvement, is present in about 5% of cases.
- Hepatosplenomegaly: the enlargement of both the liver and spleen caused by the same disease.
- Pain following alcohol consumption: classically, involved nodes are painful after alcohol consumption, though this phenomenon is very uncommon, occurring in only two to three percent of people with Hodgkin's lymphoma, thus having a low sensitivity. On the other hand, its specificity is high enough for it to be regarded as a pathognomonic sign of Hodgkin lymphoma. The pain typically has an onset within minutes after ingesting alcohol, and is usually felt as coming from the vicinity where there is an involved lymph node. The pain has been described as either sharp and stabbing or dull and aching.
- Back pain: nonspecific back pain (pain that cannot be localised or its cause determined by examination or scanning techniques) has been reported in some cases of Hodgkin's lymphoma. The lower back is most often affected.
- Red-coloured patches on the skin, easy bleeding and petechiae due to low platelet count (as a result of bone marrow infiltration, increased trapping in the spleen etc.—i.e. decreased production, increased removal)
- Systemic symptoms: about one-third of patients with Hodgkin's disease may also present with systemic symptoms, including low-grade fever; night sweats; unexplained weight loss of at least 10% of the patient's total body mass in six months or less, itchy skin (pruritus) due to increased levels of eosinophils in the bloodstream; or fatigue (lassitude). Systemic symptoms such as fever, night sweats, and weight loss are known as B symptoms; thus, presence of fever, weight loss, and night sweats indicate that the patient's stage is, for example, 2B instead of 2A.
- Cyclical fever: patients may also present with a cyclical high-grade fever known as the Pel-Ebstein fever, or more simply "P-E fever". However, there is debate as to whether the P-E fever truly exists.
- Nephrotic syndrome can occur in individuals with Hodgkin's lymphoma and is most commonly caused by minimal change disease
No one really knows what causes Hodgkin's disease, but we do know that it can't be caused by getting someone else's germs or by eating the wrong foods. People who have had Epstein-Barr virus, which can cause infectious mononucleosis (mono), may be at a slightly higher risk for Hodgkin's. There is a slightly increased risk of Hodgkin's among family members of patients who carry the disease.
Because the cause of Hodgkin's is still unknown, there is no special prevention, but a risk factor is anything that affects your chance of getting a disease such as cancer.
Different cancers have different risk factors. For example, exposing skin to strong sunlight is a risk factor for skin cancer. Smoking is a risk factor for cancers of the lung, mouth, larynx (voice box), bladder, kidney, pancreas, and several other organs.
But risk factors don't tell us everything. Having a risk factor, or even several, does not mean that you will get the disease. And many people who get the disease may not have had any known risk factors. Even if a person with Hodgkin disease has a risk factor, it is often very hard to know how much that risk factor may have contributed to the lymphoma.
Tests and procedures used to diagnose Hodgkin's lymphoma include:
- Physical exam. Your doctor checks for swollen lymph nodes, including in your neck, underarm and groin, as well as a swollen spleen or liver.
- Blood tests. A sample of your blood is examined in a lab to see if anything in your blood indicates the possibility of cancer.
- Imaging tests. Imaging tests used to diagnose Hodgkin's lymphoma include X-ray, computerized tomography (CT) scan and positron emission tomography (PET-scan).
- Surgery to remove a swollen lymph node. Minor surgery may be done to remove all or part of an enlarged lymph node for testing. The lymph node is sent to a laboratory for testing. A diagnosis of Hodgkin's lymphoma is made if the abnormal Reed-Sternberg cells are found within the lymph node.
- A procedure to collect bone marrow for testing. A bone marrow biopsy may be used to look for signs of cancer in the bone marrow. During this procedure, a small amount of bone marrow, blood and bone are removed through a needle.
After your doctor has determined the extent of your Hodgkin's lymphoma, your cancer will be assigned a stage. Your cancer's stage helps determine your prognosis and your treatment options.
Stages of Hodgkin's lymphoma include:
- Stage I. The cancer is limited to one lymph node region or a single organ.
- Stage II. In this stage, the cancer is in two lymph node regions or the cancer has invaded one organ and the nearby lymph nodes. But the cancer is still limited to a section of the body either above or below the diaphragm.
- Stage III. When the cancer moves to lymph nodes both above and below the diaphragm, it's considered stage III. Cancer may also be in one portion of tissue or an organ near the lymph node groups or in the spleen.
- Stage IV. This is the most advanced stage of Hodgkin's lymphoma. Cancer cells are in several portions of one or more organs and tissues. Stage IV Hodgkin's lymphoma affects not only the lymph nodes but also other parts of your body, such as the liver, lungs or bones.
Additionally, your doctor uses the letters A and B to indicate whether you're experiencing symptoms of Hodgkin's lymphoma:
- A means that you don't have any significant symptoms as a result of the cancer.
- B indicates that you may have significant signs and symptoms, such as a persistent fever, unintended weight loss or severe night sweats.
The prognosis (chance of recovery) and treatment options depend on the following:
- The stage of the cancer.
- The size of the tumor and how quickly it shrinks after initial treatment.
- The patient's symptoms when diagnosed.
- Certain features of the cancer cells.
Whether the cancer is newly diagnosed, does not respond to initial treatment, or has recurred (come back).
Patients with early stage disease (IA or IIA) are effectively treated with radiation therapy or chemotherapy. The choice of treatment depends on the age, sex, bulk and the histological subtype of the disease. Adding localised radiation therapy after the chemotherapy regimen controls the tumors better and provides a better chance for survival than chemotherapy alone. Patients with later disease (III, IVA, or IVB) are treated with combination chemotherapy alone. Patients of any stage with a large mass in the chest are usually treated with combined chemotherapy and radiation therapy.
It should be noted that the common non-Hodgkin's treatment, rituximab (which is a monoclonal antibody against CD20) is not routinely used to treat Hodgkin's lymphoma due to the lack of CD20 surface antigens in most cases. The use of rituximab in Hodgkin's lymphoma, including the lymphocyte predominant subtype has been reviewed recently.
Although increased age is an adverse risk factor for Hodgkin's lymphoma, in general elderly patients without major comorbidities are sufficiently fit to tolerate standard therapy, and have a treatment outcome comparable to that of younger patients. However, the disease is a different entity in older patients and different considerations enter into treatment decisions.
For Hodgkin's lymphomas, radiation oncologists typically use external beam radiation therapy (sometimes shortened to EBRT or XRT). Radiation oncologists deliver external beam radiation therapy to the lymphoma from a machine called linear accelerator which produces high energy X Rays and Electrons. Patients usually describe treatments as painless and similar to getting an X-ray. Treatments last less than 30 minutes each.
For lymphomas, there are a few different ways radiation oncologists target the cancer cells. Involved field radiation is when the radiation oncologists give radiation only to those parts of the patient's body known to have the cancer. Very often, this is combined with chemotherapy. Radiation therapy directed above the diaphragm to the neck, chest and/or underarms is called mantle field radiation. Radiation to below the diaphragm to the abdomen, spleen and/or pelvis is called inverted-Y field radiation. Total nodal irradiation is when the therapist gives radiation to all the lymph nodes in the body to destroy cells that may have spread.
The high cure rates and long survival of many patients with Hodgkin's lymphoma has led to a high concern with late adverse effects of treatment, including cardiovascular disease and second malignancies such as acute leukemias, lymphomas, and solid tumors within the radiation therapy field. Most patients with early-stage disease are now treated with abbreviated chemotherapy and involved-field radiation therapy rather than with radiation therapy alone. Clinical research strategies are exploring reduction of the duration of chemotherapy and dose and volume of radiation therapy in an attempt to reduce late morbidity and mortality of treatment while maintaining high cure rates. Hospitals are also treating those who respond quickly to chemotherapy with no radiation.