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T-Cell Lymphoblastic Lymphoma (T-LBL)
Stages of LBL
Adults
Pediatrics
The staging system most often used to describe the spread of non-Hodgkin's lymphoma in adults, including lymphoblastic lymphoma, is called the Ann Arbor staging system. The stages are described by Roman numerals I through IV (1-4). Lymphomas that affect organs outside of the lymph system (“extranodal” organs) have “E” added to their stage (for example, stage IIE).
Stage I: Either of the following means the disease is a stage I:
- The lymphoma is in a lymph node or nodes in only 1 region, such as the neck, groin, underarm, and so on
- The cancer is found in only 1 area of a single organ outside of the lymph system (IE)
Stage II: Either of the following means the disease is a stage II:
- The lymphoma is in 2 groups or more of lymph nodes on the same side of (above or below) the diaphragm (the breathing muscle that aids breathing and separates the chest and abdomen). For example, this might include nodes in the underarm and neck area but not the combination of underarm and groin nodes
- If the cancer extends locally from a single group of lymph node(s) into a nearby organ, it is called IIE
Stage III: Either of the following means the disease is a stage III:
- The lymphoma is found in lymph node areas on both sides of (above and below) the diaphragm
- The cancer may also have extended into an area or organ next to the lymph node (IIIE), into the spleen (IIIS), or both (IIISE)
Stage IV: Any of the following means the disease is a stage IV:
- The lymphoma has spread outside of the lymph system into an organ that is not immediately adjacent to an involved node
The letter “B” is added (stage IIIB, for example) if any B symptoms are present:
- unexplained weight loss (more than 10% of weight)
- soaking night sweats
- unexplained fever >100°
For patients without these symptoms, the letter “A” is added to their stage.
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Pediatrics: [21]
The staging system most often used to describe the spread of non-Hodgkin's lymphoma in children, including lymphoblastic lymphoma, is called the St. Jude staging system.
Stage I:
Non-Hodgkin's lymphoma starting in one place, either as a single tumor not in lymph nodes or in lymph nodes in one part of the body (the neck, groin, underarm, etc.). The lymphoma is not in the chest or abdomen.
Stage II:
In Stage II, the lymphoma is not in the chest. There is a single tumor mass not in lymph nodes that has spread to one nearby group of lymph nodes.
Or
The lymphoma started in the intestinal tract and all visible tumor masses have been removed.
Or
The lymphoma is growing as 2 separate tumors not in lymph nodes or in more than one group of lymph nodes where they are on the same side of (above or below) the diaphragm (the breathing muscle that separates the chest and abdomen). For example, this might mean nodes in the underarm and neck area are affected but not the combination of underarm and groin nodes.
Stage III:
This includes any non-Hodgkin's lymphoma that starts in the chest (usually in the thymus or lymph nodes in the center of the chest or the lining of the lung)
Or
Lymphoma starting in the abdomen that has spread widely within the abdomen and cannot be completely removed by surgery
Or
Lymphoma located next to the spine
Or
The lymphoma is in 2 groups of lymph nodes on different sides of the diaphragm
Or
Two lymphoma tumors, not in lymph nodes, that are on different sides of the diaphragm
Stage IV:
This includes any non-Hodgkin's lymphoma that has spread to the bone marrow (but less than 25% of the cells are lymphoma) or the central nervous system (brain or spinal fluid).
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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.
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