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Lymphoblastic Lymphoma

  • Lymphoblastic lymphoma is a type of non-Hodgkin lymphoma, a cancer in the lymphatic system. The lymphatic system is part of the immune system, and functions to fight disease and infections. Most cases of lymphoblastic lymphoma involve the T-cells in the thymus, and usually become evident with a mass in the chest and swollen lymph nodes.

    • It tends to spread quickly to the bone marrow, lymph nodes, and sometime, to the surface of the brain and the membranes that surround the lungs and heart.
    • It’s thought to be caused by genetics and/or exposure to viral infections, radiation, or chemotherapy.
    • It accounts for about 35 percent of lymphomas in children.
    • It’s more common in Caucasian boys.
    • Treatments may involve a combination of therapies including surgery, radiation, and/or chemotherapy.

    How Dana-Farber/Boston Children's approaches lymphoblastic lymphoma

    Patients with lymphoblastic lymphoma are treated through the Lymphoma Program at 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. Our multidisciplinary approach to care ensures thoughtful discussion of every treatment decision and individualized care plans for each patient. Our team integrates expertise from the following specialists:

    • pediatric oncologists, pediatric hematopathologists (pathologists specializing in childhood blood diseases including leukemia and lymphoma), surgical oncologists, and radiation oncologists
    • experts from every pediatric medical subspecialty, including diagnostic and interventional radiology, nuclear medicine, cardiology, endocrinology, and pediatric anesthesia, among others
    • highly skilled and experienced pediatric oncology nurses
    • child life specialists, psychologists, social workers and resource specialists who provide supportive care before, during and after treatment

    Scientists at Dana-Farber/Boston Children's Cancer and Blood Disorders Center are also conducting numerous research studies that will help clinicians better understand and treat lymphoblastic lymphoma.

  • What is lymphoblastic lymphoma?

    Lymphoblastic lymphoma is a type of non-Hodgkin lymphoma, a cancer in the lymphatic system. The lymphatic system is part of the immune system, and functions to fight disease and infections. Most cases of lymphoblastic lymphoma involve the T-cells in the thymus, and usually become evident with a mass in the chest and swollen lymph nodes. It tends to spread quickly to the bone marrow, lymph nodes, and sometime, to the surface of the brain and the membranes that surround the lungs and heart.

    What causes lymphoblastic lymphoma?

    The specific cause of non-Hodgkin lymphoma is unclear. Some theories include:

    • genetics
    • exposure to viral infections such as Epstein-Barr virus and human immunodeficiency virus (HIV)
    • exposure to chemotherapy and radiation therapy
    • hereditary abnormalities such as ataxia telangiectasia, X-linked lymphoproliferative disease, or the Wiskott-Aldrich syndrome

    Is lymphoblastic lymphoma common?

    Lymphoblastic lymphoma accounts for about 35 percent of lymphomas in children. It’s most common in teenagers; boys are affected twice as often as girls. Non-Hodgkin lymphoma as a group is the third most common childhood cancer. More specifically, non-Hodgkin lymphoma:

    • occurs most often in children between the ages of 7 and 11, but can occur at any age from infancy to adulthood.
    • is more common among Caucasian children than among African-American children and children of other races.

    What are the symptoms of lymphoblastic lymphoma?

    The disease can progress quickly from a few days to a few weeks. Your child can go from otherwise healthy to having multi-system involvement in a short time period. While symptoms may vary child-to-child, the most common include:

    • respiratory problems, pain with deep breaths (dyspnea), cough, and/or wheezing.
    • abdominal pain
    • constipation
    • 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 (fatigue)
    • weight loss/decreased appetite
    • itching of the skin
    • recurring infections
  • How does a doctor know that it’s lymphoblastic lymphoma?

    Diagnostic procedures for lymphoblastic lymphoma are used to determine the exact type of tumor your child has and whether the tumor has spread. These may include a:

    • Physical exam, including neurologic function tests including: reflexes, muscle strength, eye and mouth movement, coordination and alertness.
    • X-rays to produce images of internal tissues, bones, and organs onto film.
    • Computerized tomography scan (also called a CT or CAT scan) to capture a detailed view of the body, in some cases.
    • Biopsy or tissue sample from the tumor to provide definitive information about the type of tumor; this is collected during surgery.
    • Lymphangiogram, an imaging study that can detect cancer cells or abnormalities in the lymphatic system and structures; it involves a dye being injected into the lymph system.
    • Blood and urine tests
    • Bone marrow aspiration/biopsy is 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 (spinal tap) to remove a small sample of cerebrospinal fluid (CSF) and determine if any tumor cells have started to spread. In young children, this procedure is safely performed under sedation, and is less difficult and less painful than placing an intravenous (IV) catheter.
  • Traditional treatments for lymphoblastic lymphoma

    Treatments for lymphoblastic lymphoma may involve a combination of therapies including:

    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 compresses the airway and/or the 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

    Our doctors use precisely targeted and dosed radiation therapy to kill cancer cells left behind after your child's surgery.

    Chemotherapy

    Chemotherapy is a drug that interferes with the cancer cell's ability to grow or reproduce.

    • Different groups of chemotherapy drugs work in different ways to fight cancer cells and shrink tumors.
    • Often, a combination of chemotherapy drugs is used.
    • Certain chemotherapy drugs may be given in a specific order depending on the type of cancer it is being used to treat.

    While chemotherapy can be quite effective in treating certain cancers, the agents 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 the care team, parents, and child prepare, and, in some cases, prevent these symptoms from occurring, if possible.

    Chemotherapy is systemic treatment, meaning it is introduced to the bloodstream and travels throughout the body to kill cancer cells. Chemotherapy can be given:

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

    Supportive Care

    This includes any type of treatment to prevent and treat infections, side effects of treatments, and complications, and to keep your child comfortable during treatment.

    What is the recommended long-term care for children treated for lymphoblastic lymphoma?

    Children treated for lymphoblastic lymphoma should visit a cancer survivorship clinic every year to:

    • manage disease complications
    • screen for early recurrence of cancer
    • manage late effects of treatment

    A typical follow-up visit may include some or all of the following:

    • a physical exam
    • laboratory testing
    • imaging scans

    Through the David B. Perini, Jr. Quality of Life Clinic at Dana-Farber/Boston Children's Cancer and Blood Disorders Center, childhood cancer survivors receive a comprehensive follow-up evaluation from their cancer care team.

    • Our childhood cancer survivorship clinic is held weekly.
    • In addition to meeting with your pediatric oncologists, your child may see one of our endocrinologists, cardiologists, neurologists, neuro-psychologists or alternative/complementary therapy specialists.
    • We also offer the following services:
      • patient and family education
      • psychosocial assessment
      • genetic counseling
      • reproductive and fertility evaluation and counseling
      • opportunities to speak with other childhood cancer survivors
    Comfort for lymphoma patients and their families

    The Pediatric Stem Cell Transplantation Program at Dana-Farber/Boston Children's heavily emphasizes safety and cleanliness to help protect patients with lymphoma, and other conditions undergoing bone marrow and stem cell transplantation. For example, families and clinicians have to clean themselves before being entering the unit. Dana-Farber/Boston Children's understands that this can make families anxious, and have made improvements to make the hospital more comfortable for families, such as providing beds for parents and families to sleep over so they can provide emotional support for their child.

  • The Dana-Farber/Boston Children’s researchers are active members 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.

    Research underway

    The Dana-Farber/Boston Children’s Stem Cell Transplant Center is one of only eight institutions around the country that is investigating the use of umbilical cord transplantation.

    Also under study is a new method for preventing graft versus host disease, a serious complication that occurs when transplanted cells do not recognize the tissues and organs of your child’s body and react against the your child’s tissue. The result of this treatment approach, if it continues to be as successful, will be that the degree of match between donor and the recipient will not need to be particularly close, greatly increasing the pool of potential donors for each patient. This could also eliminate the need for long-term drug therapy traditionally needed to treat graft versus host disease.

    Other types of treatment currently being studied include:

    • Angiogenesis inhibitors are substances that may be able to prevent the growth of tumor.
    • Biological therapies use a wide range of substances that may be able to involve the body's own immune system to fight cancer or lessen harmful side effects of some treatments.
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