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T-Cell Lymphoblastic Lymphoma (T-LBL)
Causes & Risk Factors [14]
Lymphoblastic lymphoma (LBL) is a rare but aggressive form of non-Hodgkin’s Lymphoma (NHL). The exact cause of most cases of NHL is not known. Most patients with NHL do not have any known risk factors, and therefore, the causes of their cancers remain unknown. However, there are certain conditions and risk factors that may lead to the development of this cancer type.
Age is the greatest risk factor for this disease. Most cases are found in people in their 60s. In addition, being obese might increase the risk of NHL. [14]
An abnormal or deficient immune system may make a person more likely to develop NHL. Therefore, patients with HIV and those with transplanted organs or other cancers who are receiving chemotherapy  and/or radiation may be at higher risks of developing NHL. The congenital immunodeficiency syndromes that have been associated with an increased incidence of NHL include:
- Wiskott-Aldrich syndrome
- Severe combined immunodeficiency syndrome (SCID)
- Ataxia-telaniectasia
- Common variable immunodeficiency
- Bloom's syndrome
- X-linked lymphoproliferative syndrome
DNA mutations can also cause NHL; however, they usually occur during a patient’s life rather than having been inherited before birth as with other cancers. These mutations may result from exposure to radiation or cancer-causing chemicals, but sometimes they occur for no apparent reason. Translocations can also be involved in some cases of NHL.
Other infections such as malaria and the Epstein-Barr (EB) virus can also cause NHL to develop. In patients with T-cell deficiency, the EB virus-infected B cells grow and accumulate. These growing cells have an increased risk for developing DNA mutations or gene translocations. If these changes affect certain oncogenes or tumor suppressor genes, lymphoma will develop. Recent reports have suggested that infection with the hepatitis C virus might be a risk factor for NHL.
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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