Lymphomas

ALSO KNOWN AS: Hodgkin disease, non-Hodgkin lymphoma, Burkitt lymphoma

RELATED CONDITIONS: Cancer of the lymph nodes, cancer of the spleen, leukemia, (AIDS)

DEFINITION: Lymphatic cancer is a blood cancer that involves lymphocytes (white blood cells). Cancerous cells grow and multiply, mainly in the lymph nodes and spleen, where they cause swelling and suppression of the body’s natural immune system. Lymphoma occurs as Hodgkin disease and non-Hodgkin lymphoma. The presence of abnormal cells, known as Reed-Sternberg cells, named after the scientists who discovered them, indicates Hodgkin disease and differentiates this lymphoma from all other types, including Burkitt lymphoma, designated non-Hodgkin lymphoma.

Risk factors: As the causes of Hodgkin disease and non-Hodgkin lymphoma are unknown, the risk factors are uncertain. However, in non-Hodgkin lymphoma, the suppression of the immune system is a significant risk factor, particularly in high-risk patients like organ transplant recipients taking antirejection medications. People who have had the human immunodeficiency virus (HIV) for four or more years are also at increased risk.

A link has been detected between the development of lymphoma and exposure to flour in some agricultural jobs. In more advanced cases of the disease, a link has been found to exposure to X-rays and certain forms of chemotherapy. Heredity has little effect on the development of lymphomas.

Etiology and the disease process: The causes of lymphoma are not fully understood. Because it is not a single disease with clear-cut boundaries, it is difficult to understand and assess. Some lymphomas are relatively easy to treat and have reasonable survival rates, but other forms grow rapidly and aggressively, making successful treatment more problematic. In the United States, lymphoma occurs most often among the well-educated and those in more affluent socioeconomic situations.

The lymphatic system contains B cells and T cells. The former manufacture antibodies designed to fight infections, and T cells regulate the immune system. More than 90 percent of lymphomas in the United States originate in B cells. Lymphatic cancer cells can be present in the stomach, intestines, bones, skin, sinuses, and lymph nodes.

Incidence: Hodgkin disease and non-Hodgkin lymphoma are found more often in men than in women. Non-Hodgkin lymphoma affects more people in their twenties and in the fifty-five-to-seventy age group. One variety of non-Hodgkin lymphoma, Burkitt lymphoma, is mainly found in the tropics and Africa. It is thought to be related in some way to the Epstein-Barr virus.

Symptoms: The most frequent symptom is a swelling in the lymph nodes in the neck, under the arms, or in the groin, usually referred to as swollen glands. In some cases, particularly in young children, the thymus gland in the upper chest may also be swollen.

The swelling is visible in most cases and usually is not painful. It is sometimes accompanied by other symptoms, such as loss of appetite, fever, weight loss, and night sweats, which are frequently mistaken for influenza. These symptoms may disappear after a short time only to reappear.

Patients with lymphoma often experience an overall feeling of illness characterized by lethargy, headaches, and skin ulceration accompanied by itching. Pain, bleeding, and swelling may occur if the disease spreads to the abdomen. In such cases, the patient may vomit blood or have blackened stools, indicating internal bleeding.

Because non-Hodgkin lymphoma usually grows slowly, it may be asymptomatic or may produce only minor symptoms that can easily be ignored in the early stages of the disease. Therefore, this type of lymphoma is frequently diagnosed at Stage III or IV rather than early stages when the cure rate is greatest. Hodgkin disease grows and spreads rapidly. Its early symptoms may cause patients to seek medical intervention in the earlier stages of the disease.

Screening and diagnosis: The usual method for diagnosing lymphoma involves the removal and microscopic examination of tissue from the lymph nodes for biopsy. If cancer cells are found, further diagnosis may be indicated involving X-rays of the chest or lymph glands, removal of bone marrow to be biopsied, ultrasound, and scanning by computed (CT), (MRI), or positron emission tomography (PET).

The Lugano classification system, a modification of the Ann Arbor staging system, is used for Hodgkin disease and non-Hodgkin lymphoma:

• Stage I: Cancer cells have been found in only one section of the lymph nodes or in just one confined area outside the lymph nodes.
• Stage II: Cancer has been detected in two or more lymph nodes on the same side of the diaphragm or if cancer cells are found in one group of lymph nodes and a nearby organ.
• Stage III: Cancer cells are on both sides of the diaphragm or above the diaphragm and in the spleen.
• Stage IV: Cancer cells are found in at least one organ outside the lymphatic area.

Treatment and therapy: A determines whether cancer cells are present and can specifically identify the kinds of cancer cells. It is important to customize treatment for each patient to the greatest degree possible. Therefore, identification of the precise kinds of cancerous cells is vital.

For patients with Stage I and Stage II lymphomas, the first avenue of treatment is radiation therapy. If there are signs that the malignancy has spread, radiation may be supplemented by chemotherapy. If the disease has advanced considerably or is likely to, the treatment of choice may be a bone marrow transplant.

Bone marrow transplants—considered a last resort treatment—are often combined with continued radiation therapy and chemotherapy. These patients usually have a severely compromised immune system, so the postoperative treatment involves isolation under sterile conditions for an extended period to prevent infection from opportunistic diseases found in hospitals.

An alternative treatment is the mini bone marrow transplant. This method involves using low-level, minimally toxic chemotherapy or radiation therapy to kill some of the patient’s bone marrow, leaving some cancer cells. Cancer-free bone marrow from a donor is introduced into the patient’s bone marrow. In time, this cancer-free bone marrow produces cancer-free cells that attack and destroy the remaining cancerous cells. Stem cell research also shows considerable promise in treating lymphoma.

Prognosis, prevention, and outcomes: Avoiding some occupational and environmental hazards may decrease the likelihood of developing lymphoma. Links have been made between increases in the spread of the disease and environmental hazards like hydrocarbons and toxic fumes. Avoiding polluted air and water is undoubtedly essential.

Also, diet has profound effects in combating many forms of cancer, including lymphoma. A diet low in fats, limited in animal protein, and containing few refined carbohydrates will strengthen the immune system and improve overall well-being. People should routinely eat at least five servings of fruits and vegetables daily.

The outlook for lymphoma patients depends on the stage at which the cancer was detected. Many Stage I patients are cured of the disease. The five-year survival rate among Stage I and Stage II patients is between 74 and 88 percent. Among Stage IV patients, the two-year survival rate is about 50 percent. Each year, the statistics are more encouraging as new medications and techniques are developed and employed in treating the disease.

Early diagnosis is key to survival. Any symptoms should receive the attention of a qualified physician. Particularly dangerous are phantom symptoms—those that disappear quickly but then return. The interval between their first appearance and their return is crucial because this is the period in which the disease is most susceptible to treatment.

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