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Burkitt's Lymphoma

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    Burkitt's lymphoma (also called small noncleaved cell lymphoma) is a type of non-Hodgkin lymphoma, a cancer in the lymphatic system.

    • Non-Hodgkin lymphoma causes the cells in your child’s lymphatic system to abnormally reproduce, eventually causing tumors to grow.

    • Non-Hodgkin disease cells can also spread to other organs and tissues in the body.

    • Non-Hodgkin lymphoma is the third most common childhood cancer.

    • It occurs most often in children between the ages of 7 and 11, but can occur at any age from infancy to adulthood.

    • Non-Hodgkin lymphoma affects males almost three times more often than females, and is more common among Caucasian children than among African-American children and children of other races.

    Patients with Burkitt's lymphoma are treated through Dana-Farber/Boston Children's Cancer and Blood Disorders Center, an integrated pediatric hematology and oncology partnership between Dana-Farber Cancer Institute and Boston Children’s Hospital. We utilize the expertise of both Boston Children's Hospital, ranked the #1 children's hospital in the country, and the Dana-Farber Cancer Institute, a nationally recognized leader in cancer care and a member of the Dana-Farber/Harvard Cancer Center. Members of our physician team all have specific expertise in pediatric lymphomas. Specific disciplines include oncology, radiation oncology, surgery, interventional and diagnostic radiology, and hematopathology. We treat all forms of non-Hodgkin lymphoma and related disorders.

    Children's Hospital Boston
    300 Longwood Avenue (Inpatient)
    44 Binney Street, 3rd Floor (Outpatient)
    Boston MA 02115

    call: 888-PEDI-ONC
    fax: (617) 632-4248

  • What is the lymphatic system?

    The lymphatic system is part of the immune system and functions to fight disease and infections. The lymphatic system includes:

    •  lymphocytes- specific white blood cells that fight infections and disease. The two main types of lymphocytes are B-cells and T-cells. Normal B-cells mature into plasma cells and produce immunoglobulins (also called antibodies) that help protect the body from bacteria. Normal T-cells serve in several roles to help protect the body. They recognize and destroy virus-infected cells, and they help attract other white blood cells that kill infected cells. There are several types and different stages of maturation for these cells.
    • lymph fluid- fluid containing lymphocyte cells
    • lymph vessels- thin tubes that carry lymph fluid throughout the body, like blood vessels
    • lymph nodes- bean-shaped organs, found in the underarm, groin, neck, chest, and abdomen, that act as filters for the lymph fluid as it circulates through your child’s body
    • spleen- a small organ that produces lymphocytes, stores healthy blood cells, and filters out damaged blood cells, bacteria and cell waste
    • thymus- a small gland, located in front of the heart, that plays a vital role in the development of T-cells while the child is still in the womb. Although the size of the gland starts to decrease after young adulthood, it continues to serve as part of the immune system throughout life.
    • bone marrow/stem cells- the bone marrow contains stem cells, cells that develop into red blood cells, platelets (clotting cells) and infection-fighting white blood cells, including lymphocytes. Stem cells are also found circulating throughout the body in the blood stream.

    How does Burkitt’s lymphoma differ from other non-Hodgkin lymphomas?

    There are several types of non-Hodgkin lymphomas, classified by physicians based on the size and shape of the lymphoma cells under a microscope, and how the cells grow within the lymph nodes and beyond.

    • Burkitt's lymphoma has what are considered small cells that are “noncleaved,” meaning the have no folds or indentations when viewed under a microscope.
    • Burkitt's lymphoma usually develops in the abdomen and spreads to other organs, including the brain.
    • Burkitt's lymphoma involves B-cells and is a rapidly growing cancer.
    • There is also a type of small, noncleaved lymphoma that has a somewhat different appearance under the microscope that is called non-Burkitt's lymphoma, which usually occurs in adults.

    What causes Burkitt's lymphoma?

    The specific cause of non-Hodgkin lymphoma is unclear.  Some possibilities for factors that may increased your child’s risk of developing this cancer include:

    • genetics
    • exposure to viral infections
    • Non-Hodgkin lymphoma has also been linked to chemotherapy and radiation therapy—it may be a second malignancy as a result of the treatment for certain cancers.

    There has been much investigation into the association of non-Hodgkin lymphoma with:

    Both of these infectious viruses have been linked to the development of Burkitt's lymphoma.

    The majority of Burkitt's lymphoma cases result from a chromosome rearrangement between chromosome #8 and #14, which causes genes to change positions and function differently, promoting uncontrolled cell growth.

    Other chromosome rearrangements have been seen in non-Hodgkin lymphoma (all types) are also thought to promote excessive cell growth. Children and adults with other hereditary abnormalities have an increased risk of developing non-Hodgkin lymphoma, including patients with:

    • ataxia telangiectasia
    • X-linked lymphoproliferative disease
    • the Wiskott-Aldrich syndrome

    What are the symptoms of non-Hodgkin lymphoma?

    Most children have stage III or IV disease at the time of diagnosis because of the sudden onset of symptoms. The disease can progress quickly from a few days to a few weeks, and your child could go from otherwise healthy to having multi-system involvement in a short time period.

    Some children with non-Hodgkin lymphoma have symptoms of an abdominal mass and complain of abdominal pain, fever, constipation and decreased appetite (due to the pressure and obstruction a large tumor in this area can cause). Some children with non-Hodgkin lymphoma have symptoms of a mass in their chest and complain of respiratory problems, dyspnea (pain with deep breaths), cough and/or wheezing.

    Because of the rapid onset of this malignancy, any respiratory symptoms can quickly worsen, causing a life-threatening emergency.

    While each child may experience symptoms differently, some of the most common include: 

    • painless swelling of the lymph nodes in neck, chest, abdomen, underarm or groin
    • fever
    • sore throat
    • fullness in groin area from node involvement
    • bone and joint pain
    • night sweats
    • tiring easily
    • weight loss/decreased appetite
    • itching of the skin
    • recurring infections

    The symptoms of non-Hodgkin lymphoma may resemble other blood disorders or medical problems. Always consult your child's physician for a diagnosis.

  • Tests

    How is non-Hodgkin lymphoma diagnosed?

    In addition to a complete medical history and physical examination, diagnostic procedures for non-Hodgkin lymphoma may include:

    • blood and urine tests
    •  x-raysof the chest- a diagnostic test which uses invisible electromagnetic energy beams to produce images of your child’s internal tissues, bones, and organs onto film
    • lymph node biopsy- a sample of tissue is removed from a lymph node and examined under a microscope
    •  computerized tomography scan(also called a CT or CAT scan) - a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat and organs. CT scans are more detailed than general x-rays.
    • lymphangiogram (LAG)- Dye is injected into lymphatic system to determine how much it is involved in areas that are otherwise difficult to visualize.
    • bone marrow biopsy/aspiration -a procedure that involves a small amount of bone marrow fluid and tissue to be taken, usually from part of the hip bones, to further examine the number, size, and maturity of blood cells and/or abnormal cells.
    • lumbar puncture (to evaluate central nervous system disease for cancer cells) - a special needle is placed into your child’s lower back, into the spinal canal. This is the area around the spinal cord. A small amount of cerebral spinal fluid (CSF) can be removed and sent for testing. CSF is the fluid that bathes the brain and spinal cord.

    How is non-Hodgkin lymphoma staged?

    Staging is the process of determining whether cancer has spread and, if so, how far. There are various staging symptoms that are used for non-Hodgkin lymphoma. Always consult your child's physician for information on staging.

    One method of staging non-Hodgkin lymphoma is the following:

    • stage I - involves the tumor at one site, either nodal or elsewhere in the body
    • stage II - involves the tumor at two or more sites on the same side of the body
    • stage III - involves tumors in any number that occur on both sides of the body, but does not involve bone marrow or the central nervous system
    • stage IV - is any stage of tumor that also has bone marrow and/or central nervous system involvement. Stage IV is also subdivided depending on the amount of blasts (cancer cells) present in the bone marrow.
  • What is the treatment for Burkitt's lymphoma?

    Specific treatment for Burkitt's lymphoma will be determined by your child's physician based on:

    • your child's age, overall health and medical history
    • the extent of the disease
    • your child's tolerance for certain medications, procedures or therapies
    • how your child's physician expects the disease to progress
    • your opinion or preference

    Treatment components may include the following (alone or in combination):

    Chemotherapy

    Chemotherapy is a drug treatment that works by interfering with the cancer cell's ability to grow or reproduce. Because Burkitt's lymphoma grows so quickly, chemotherapy treatment may be intense and last 6-8 months. Treatment may include drugs given directly to central nervous system (CNS) through the blood stream or a small puncture in the spine to prevent cancerous cells from multiplying in the spinal cold or brain.

    How is chemotherapy given?

    Different chemotherapies may be given:

    • as a pill to swallow
    • as an injection into the muscle or fat tissue
    • intravenously (directly to the bloodstream, also called IV)
    • intrathecally (directly into the spinal column with a needle)

    How is chemotherapy used?

    This depends on many factors.  Some things to keep in mind:

    • A number of combinations of chemotherapy drugs are currently being used to stabilize and, in some cases, to shrink the size of the tumor.
    • Depending on the type of cancer, chemotherapy drugs may be given in a specific order.
    • Chemotherapy may be used alone for some types of cancer, or in conjunction with other therapy such as radiation or surgery.

    Does chemotherapy come with bad side effects?

    While chemotherapy can be quite effective, the drugs do not differentiate normal healthy cells from cancer cells. Because of this, there can be many adverse side effects during treatment. Being able to anticipate these side effects can help your child, family and your child's health care team prepare for and sometimes prevent these symptoms from occurring.

    Surgery

    Surgery usually plays a limited role in the treatment of lymphoma. In some instances, a child may need to have a tumor removed. This may be the case if, for example, the tumor originates in the mediastinum region, where it may compress your child's airway and/or heart and major vessels. Surgery in this area may entail considerable risk, and must be carefully orchestrated between the surgeon, oncologist and anesthesiologist.

    Radiation therapy

    Radiation therapy uses high-energy rays (radiation) from a specialized machine to damage or kill cancer cells and shrink tumors. This type of therapy is most effective when there is a bulky, localized tumor that will not respond completely to chemotherapy.

    Stem cell transplant

    Stem cells are a specific type of cell from which all blood cells develop. They can develop into red blood cells to carry oxygen, white blood cells to fight disease and infection, and platelets to aid in blood clotting. Stem cells are found primarily in the bone marrow, but some also circulate in the blood stream.

    • Treatment may begin with high-dose chemotherapy (perhaps combined with radiation therapy) to destroy all of the bone marrow, stem cells and cancerous cells, in your child's body.
    • Then, healthy stem cells collected from another individual whose tissue is the same as, or almost the same as, the patients are used. This is called allogeneic transplantation. This is the most common type of stem cell transplant used to treat lymphoblastic lymphoma. Often, stem cell transplant is most appropriate for patients who have recurrent or drug-resistant disease.
    • Sometimes, your child's own stem cells are collected prior to intensive treatment and used to supplement recovery after a particularly aggressive course of treatment. Through this procedure, stem cells are taken, treated in a laboratory to kill any remaining cancer cells, and kept in frozen storage for later use. After chemotherapy and/or radiation therapy, the saved stem cells are thawed and given back to grow new bone marrow and other stem cells. This is known as autologous transplantation.

    Supportive care

    Medications including antibiotics may be given if your child experiences pain, fever, infection and nausea and/or vomiting.

    Continuous follow-up care

    Your child's health will be closely monitored to determine response to treatment, detect recurrent disease and manage late effects of treatment.

    What is the long-term outlook for a child with Burkitt's lymphoma?

    Your child's prognosis greatly depends on:

    • the extent of the disease
    • presence or absence of metastasis
    • response to therapy
    • your child's age and overall health
    • tolerance of specific medications, procedures or therapies
    • new developments in treatment

    As with any cancer, prognosis and long-term survival can vary greatly. Prompt medical attention and aggressive therapy are important for the best prognosis, and continuous follow-up care is essential. New methods are continually being discovered to improve treatment and decrease side effects of the treatment for the disease.

    What is the latest research on Burkitt's Lymphoma?

    A variety of chemotherapeutic regimens have been evaluated in the treatment of newly diagnosed high-grade gliomas.

    • While studies in adults have suggested that procarbazine, vincristine and CCNU (PVC) produce modest responses in grade III gliomas, this has not been demonstrated for pediatric cases or for grade IV tumors.
    • Several other regimens have also produced responses, but none has improved survival.
    • Increased doses of chemotherapy in the setting of autologous bone marrow transplant have also not produced notable advantage.

    Dana-Farber/Boston Children's is part of the Children's Oncology Group, a national group of pediatric oncologists whose work includes investigational treatments for newly diagnosed patients with non-Hodgkin lymphoma and those who experience relapse. Studies also seek to improve the supportive care of patients undergoing treatment. One area of treatment under investigation is biological therapy or immunotherapy, the use of drugs that seem to slow the growth of cancer cells while stimulating and strengthening the body's own immune system. Improvements are also occurring in the area of stem cell transplant, where researchers are finding better ways to improve pre-transplant treatment, broaden the range of compatible donors, and more effectively remove cancerous cells from patient's own bone marrow.

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