Lymphomas
LYMPHOMAS
The lymphatic system consists of drainage vessels which pervade every organ of the body, like the circulation. In addition, it contains collections of cells in locations such as the lymph nodes, spleen and tonsils, as well as internal lymph organs in the chest, abdomen and elsewhere. Cancer may arise in any of these areas. The commonest form of lymph node cancer is termed Hodgkin’s Disease, which constitutes about 40% of all lymph cancers; the so-called non-Hodgkin’s lymphomas comprise the rest.
Hodgkin’s Disease
Almost 8000 cases of this disease occur yearly in the U.S., with a slight male predominance. The treatment of this disease has been one of the most promising areas in cancer medicine. Despite many theories as to the cause of Hodgkin’s Disease, such as infections with viruses or other organisms, it still must be considered of unknown cause.
SYMPTOMS
Common initial symptoms consist of enlargement of lymph nodes in the neck, armpit, or elsewhere. This is usually painless, and noticed quite incidentally. Of course many benign diseases can also cause temporary enlargement of nodes, but if the enlargement lasts more than two weeks or so, suspicion must increase. In some cases this is accompanied by weight loss, fever, sweating, and itching.
DIAGNOSIS
When readily accessible, lymph nodes can be easily biopsied under local anesthesia, and often confirm the diagnosis. A chest x-ray will often show enlargement of internal lymph nodes of which the patient may have been unaware. A typical cell called a Reed-Sternberg cell is virtually diagnostic of Hodgkin’s under the microscope; one cause of similar changes is a rare reaction to the drug phenytoin or Dilantin. Other than anemia and other nonspecific blood test findings, other tests are more useful for establishing the stage of the disease than for diagnosis. Defects in the immune system predispose patients to unusual viral and parasitic infections.
Staging and classification of the various types of Hodgkin’s Disease have importance in determining treatment and prognosis. Stage I involves a single lymph node region only. Stage II involves more than one lymph node region but both on the same side of the diaphragms, or one node area and one site of spread outside the lymph system. Stage III is when node regions on both sides of the diaphragm are involved, with or without a single non-lymph organ involved. Stage IV involves diffuse spread to non-lymphatic organs. In addition different categories are assigned on the type of microscopic appearance of the cells.
TREATMENT and PROGNOSIS
Stage I and II disease usually is treated primarily with radiation therapy either alone or combined with chemotherapy. Megavoltage radiation available at referral centers is the technique of choice. The decisions of how much to give, what organs to include and so forth are crucial and individually determined. Side effects include suppression of the bone marrow, inflammation of the lung and thyroid failure.
Most stage III and IV disease is treated with chemotherapy which may include drugs such as vincristine, prednisone, procarbazine, doxorubicin, and methotrexate in various combinations given in intermittent doses over many months. Many variations and other drugs are used in various centers, and patterns are changing almost weekly.
The exciting aspect of all this is that even patients with advanced stages of the disease stand a realistic chance of being cured of the disease. For example, many regimens routinely bring about complete remissions in 70 or 80% of patients; the median duration of survival after such programs is now over 2 years and many patients are thriving ten or more years after treatment, which must be considered probable cures. Although staging does affect the prognosis within these figures, there is sufficient overlap that no patient should be considered uncurable until all efforts have been exhausted. Major referral centers and hematologists or oncologists (cancer specialists) are the consultants of choice.
OTHER LYMPHOMAS
Whereas Hodgkin’s Disease affects most patients in their 20’s to 40’s, other lymphomas strike a slightly older population on the average. Enlarged lymph nodes, fever, weight loss, sweating and fatigue are common initial symptoms. Lymphomas may also affect the nodes of the intestines more commonly, and sometimes an intestinal blockage may occur. In about 1/8 of patients, the lymph cells may pour into the blood giving a picture indistinguishable from chronic lymphocytic leukemia, discussed elsewhere.
Diagnosis is usually by surgical biopsy of an enlarged node. Like Hodgkin’s Disease, lymphomas can be staged in a complicated and technical manner, and this is important for management and prognosis. Tests often required for staging include bone marrow sampling, cat scans, and lymph system x- rays, as well as special nuclear scanning tests. Only occasionally is major surgery done for staging, as was the practice in the past.
TREATMENT and PROGNOSIS
This complex area involves a combination of surgery, radiation therapy, and chemotherapy used in various combinations or alone. It is beyond the scope of this article to detail these, but in general radiation alone is used for localized disease, surgery is primarily used for diagnosis and removal of symptomatic masses, and chemotherapy for advanced disease; the latter constitutes some 3/4 of all patients. Commonly used drugs include prednisone, chlorambucil, procarbazine, cyclophosphamide, bleomycin and doxorubicin.
Prognosis is complex, and depends as much on individual factors as it does on stage; some general survival figures show 5 year rates ranging from 50 to 75% for stage I, with 2 to 3 year median survival times for advanced disease treated with combination chemotherapy. As with many such diseases, the prognosis for patients diagnosed recently may be better, and apparent cures are seen with increasing frequency.