Richter's syndrome

Definition

Richter's syndrome is a rare and aggressive type of acute adult leukemia that results from a transformation of chronic lymphocytic leukemia into diffuse large cell lymphoma .

Description

Leukemia is a group of cancers of the white blood cells. In adults, white blood cells are made in the bone marrow of the flat bones (skull, shoulder blades, ribs, hip bones). There are three main types of white blood cells: granulocytes, monocytes, and lymphocytes. Richter's syndrome concerns only the lymphocytes.

Lymphocytic leukemia develops from lymphocytes in the bone marrow. Unlike many other cancers in which a tumor starts growing in one particular location, lymphocytic leukemia is a disease of blood cells that travel throughout the body. In chronic (long-term) lymphocytic leukemia (CLL), lymphocytes do not follow a normal life cycle, and eventually, too many will exist in the blood. They are abnormal and do not fight infections well.

In a small percentage of people, CLL, even when it is treated, transforms into a new kind of aggressive blood cancer called diffuse large cell lymphoma. When this transformation occurs, it is called Richter's syndrome. The disease is named for the American pathologist Maurice Nathaniel Richter, who practiced medicine early in the twentieth century.

Demographics

Richter's syndrome is a disease of older adults. It is an extremely rare disease. The American Cancer Society estimates that in 2000, there were 8, 100 new cases of chronic lymphocytic leukemia, and that 98% of these were in adults. Of these 8, 100 new cases, only a handful will develop into Richter's syndrome. In general, people who are more likely to get CLL are those who smoke, have been exposed to high doses of radiation, or who have had long-term exposure to herbicides and pesticides. People who have close relatives (parent, siblings or children) with CLL are also more likely to develop the disease. However, none of these risk factors predict whether CLL will develop into Richter's syndrome.

Causes and symptoms

Scientists have yet to understand why some people develop Richter's syndrome and others do not. So far, no firm genetic or environmental links have been found.

When the transformation from CLL to Richter's syndrome occurs, a change occurs in the way the lymphocytes look under the microscope. In addition, lymph nodes swell, tumors grow rapidly in the lymph system, and the patient may experience fever , night sweats , and weight loss . The patient's health deteriorates rapidly and severely.

Diagnosis

Diagnosis is made by examining blood cells under microscope and by a bone marrow biopsy . This is the same test used to diagnose CLL. A small amount of bone marrow from one of the flat bones is drawn out with a needle for laboratory examination. In some cases, lymph nodes are also removed and examined in the laboratory.

Treatment team

Since a person who develops Richter's syndrome is already a cancer patient, a treatment team is already in place. This team usually includes an oncologist (cancer specialist), a hematologist (blood specialist) and possibly a radiation oncologist (specialist in radiation therapy ), radiation or chemotherapy technicians, and nurses with special training in cancer care. With the development of Richter's syndrome, a social worker or counselor may be added to the team.

Clinical staging, treatments, and prognosis

Richter's syndrome is not staged. Chemotherapy is used to treat Richter's syndrome, although treatments are often unsuccessful. In addition, allogenic bone marrow transplantation is currently being tried in some patients. This treatment is not common and is not done at many cancer centers. For Richter's syndrome, the median survival rate (the time to which half the patients survive) is less than one year.

Alternative and complementary therapies

Alternative and complementary therapies range from herbal remedies, vitamin supplements, and special diets to spiritual practices, acupuncture, massage, and similar treatments. When these therapies are used in addition to conventional medicine, they are called complementary therapies. When they are used instead of conventional medicine, they are called alternative therapies.

There are no specific alternative therapies directed toward Richter's syndrome. However, good nutrition and activities that reduce stress and promote a positive view of life have no unwanted side effects and may help improve the quality of life.

Unlike traditional pharmaceuticals, complementary and alternative therapies are not evaluated by the United States Food and Drug Administration (FDA) for either safety or effectiveness. Patients should be wary of "miracle cures." In order to avoid any harmful side effects or interference with regular cancer treatment, patients should notify their doctors if they are using any herbal remedies, vitamin supplements, or other unprescribed treatments. Alternative and experimental treatments normally are not covered by insurance.

Coping with cancer treatment

Richter's syndrome is usually fatal within a short time. Coming to grips with this is tremendously stressful for both the patient and family members. In addition, chemotherapy treatments can cause fatigue , nausea, vomiting, and other uncomfortable side effects. Some patients decide to end treatment rather than undergo this discomfort when their chance of recovery is almost nonexistent. Others wish to continue full treatment.

This and many other personal decisions are issues to discuss with loved ones. It is often helpful for loved ones to have the support of a therapist, religious leader, or other counselor at this time when emotions are intense and often conflicting. Hospice staff members or hospital social workers or chaplains can direct patients and family members to resources that address their individual needs.

Clinical trials

As of 2001, many ongoing clinical trials related to chronic lymphocytic lymphoma may be appropriate for people with Richter's syndrome. Participation is always voluntary. The selection of clinical trials underway changes frequently. Current information on what clinical trials are available and where they are being held is available by entering the search term "chronic lymphocytic lymphoma" at the following web sites:

• National Cancer Institute. <http://cancertrials.nci.nih.gov> or (800) 4-CANCER.
• National Institutes of Health Clinical Trials. <http://clinicaltrials.gov>.
• Center Watch: A Clinical Trials Listing. <http://www.centerwatch.com>.

Prevention

There is no known way to prevent the transformation of CLL into Richter's syndrome.

Resources

PERIODICALS

Rodriguez, J., et al. "Allogenic Haematopoietic Transplantation for Richter's Syndrome." British Journal of Haematology. 110 (April 2000): 897-9.

ORGANIZATIONS

American Cancer Society. National Headquarters, 1599 Clifton Rd. NE, Atlanta, GA 30329. 800 (ACS)-2345). <http://www.cancer.org>

Leukemia & Lymphoma Society. 1311 Mamaroneck Ave., 3rd floor, White Plains, NY 10605. (800) 955-4572. <http://www.leukemia-lymphoma.org>.

Cancer Information Service, National Cancer Institute. Build ing 31, Room 10A19, 9000 Rockville Pike, Bethesda, MD 20892. (800) 4-CANCER. <http://www.nci.nih.gov/cancerinfo/index.html>.

National Leukemia Research Association, Inc. 585 Stewart Ave., Suite 536, Garden City, NY 11530. (516) 222-1944.

Tish Davidson, A.M.

KEY TERMS

Lymph nodes

—Small, bean-shaped collections of tissue found in lymph vessels. They produce cells and proteins that fight infection and filter lymph. Nodes are sometimes called lymph glands.

Lymph system

—Primary defense against infection in the body. The tissues, organs, and channels (similar to veins) that produce, store, and transport lymph and white blood cells to fight infection.

Lymphoma

—A cancer of the lymph system.

QUESTIONS TO ASK THE DOCTOR

• What kind of changes in my body can I expect to see from this cancer?
• What is the treatment plan?
• What are the likely side effects of the treatment plan?
• How long am I likely to survive?
• Since Richter's syndrome is rare, how much experience do you have with this disease?
• Are there hospitals that specialize in the treatment of Richter's syndrome where I might receive treatment unavailable here?