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T-Cell Lymphoblastic Lymphoma (T-LBL)
Treatment
The treatment of lymphoblastic lymphoma (LBL) is patterned after the treatment of acute lymphoblastic leukemia (ALL). Treatment options will vary depending on the type and location of the lymphoma as well as the stage of cancer. However, in most cases, intensive combination chemotherapy, with or without radiation, is the treatment of choice. In addition, CNS prophylaxis with methotrexate should be instituted.
Adults
The medications typically used in adults include cyclophosphamide, doxorubicin, vincristine, L-asparagine, methotrexate, prednisone, and sometimes cytosine arabinoside. Several drug combinations can be used, and treatments are usually given several times in cycles 3 to 4 weeks apart. Patients may be switched to a different drug combination if the first one does not work.
For patients who have not responded or have relapsed following at least two chemotherapy regimens, ARRANON may be a treatment option. Click here to learn  more about ARRANON.
Immunotherapy, such as interferon or monoclonal antibodies (eg, rituximab, ibritumomab tiuxetan, tositumomab), and complementary and alternative therapies may also be useful for the treatment of lymphomas. However, the benefits of using some of these options are still being researched. Stem cell transplantation may be tried when standard treatment has failed. Surgery is rarely used as a treatment option. If the lymphoma is confined to one space, radiation treatment is preferred.
Pediatrics
Typical chemotherapy regimens for children with stage I or II LBL include “CHOP” (cyclophosphamide, doxorubicin, vincristine, and prednisone) and “COMP” (cyclophosphamide, vincristine, methotrexate, and prednisone). However, the “BFM” regimen has also been used. This regimen uses a combination of 8 drugs for the first two months, followed by methotrexate and 6-mercaptopurine for up to 2 years. With either case, chemotherapy, usually given with methotrexate, is given for at least 4 doses, each separated by one week.
Children with stage III or IV LBL are often treated for up to 2 years. Treatment includes 3 phases of chemotherapy with multiple drugs and often uses the BFM regimen followed by another phase of intense treatment.
Surgery is rarely used as a treatment option, and the use of radiation is generally avoided if possible.
Recurrent LBL
Bone marrow transplantation and peripheral blood stem cell transplantation are used for children who relapse during or after treatment for LBL. Other options include treatment with a different chemotherapy regimen or enrollment in clinical trials.
Indication
ARRANON is indicated for the treatment of patients with T-cell acute lymphoblastic leukemia (T-ALL) and T-cell lymphoblastic lymphoma (T-LBL) whose disease has not responded to or has relapsed following treatment with at least two chemotherapy regimens. This use is based on the induction of complete responses. Randomized trials demonstrating increased survival or other clinical benefit have not been conducted.
Important Safety Information
ARRANON® (nelarabine) injection should be administered under the supervision of a physician experienced in the use of cancer chemotherapeutic agents. This product is for intravenous use only.
Neurologic Events: Severe neurologic events have been reported with the use of ARRANON. These events have included altered mental states including severe somnolence, central nervous system effects including convulsions, and peripheral neuropathy ranging from numbness and paresthesias to motor weakness and paralysis. There have also been reports of events associated with demyelination, and ascending peripheral neuropathies similar in appearance to Guillain-Barré syndrome.
Full recovery from these events has not always occurred with cessation of therapy with ARRANON. Close monitoring for neurologic events is strongly recommended, and ARRANON should be discontinued for neurologic events of NCI Common Toxicity Criteria grade 2 or greater.
In clinical studies of ARRANON, hematologic toxicity was the most common grade 3 (moderate) or 4 (severe) adverse event. For a complete list and incidence of adverse events: pediatric patients; adult patients. Hematologic toxicity included neutropenia, thrombocytopenia, anemia, febrile neutropenia, or neutropenia with infection.
Other common toxicities included laboratory abnormalities including increased transaminases, gastrointestinal toxicity, fatigue, and asthenia.
Patients treated previously or concurrently with intrathecal chemotherapy or previously with craniospinal irradiation may be at increased risk for neurologic adverse events.
Women of child-bearing potential should be advised to avoid becoming pregnant while receiving treatment with ARRANON.
Appropriate measures (e.g., hydration, urine alkalinization, and prophylaxis with allopurinol) must be taken to prevent hyperuricemia of tumor lysis syndrome.
Nursing should be discontinued in women who are receiving therapy with ARRANON.
Because the risk of adverse reactions to this drug may be greater in patients with severe renal impairment (CLcr <30 mL/min), these patients should be closely monitored for toxicities when treated with ARRANON.
Because the risk of adverse reactions to this drug may be greater in patients with severe hepatic impairment (bilirubin >3.0 mg/dL), these patients should be closely monitored for toxicities when treated with ARRANON.
Prescribing Information for ARRANON | Important Safety Information | Patient Information Leaflet
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