BOSTON, Sept. 7 -- The anaplastic large cell lymphoma diagnosed in Boston Red Sox rookie pitcher Jon Lester is a childhood non-Hodgkin's lymphoma (NHL), a high-grade malignancy with a favorable clinical course.
Lester said in a press briefing yesterday that he will begin a course of chemotherapy at the Dana-Farber Cancer Institute here, then return to his native Washington State for additional cycles.
Following the first cycle of chemotherapy, he will be treated at the Fred Hutchinson Cancer Research Center in Seattle.
Action Points
- Explain to interested patients that high-grade non-Hodgkin's lymphomas are generally responsive to combination chemotherapy regimens, which are more destructive to fast-growing cells such as malignancies than to normal tissues.
The Fred Hutchinson Cancer Research Center was founded by William Hutchinson, M.D., in honor of his brother, Fred, a former All-Star pitcher with the Detroit Tigers, and later manager of the Tigers, St. Louis Cardinals, and Cincinnati Reds. Fred Hutchinson died of lung cancer in 1964, at age 45.
Jon Lester's cancer, anaplastic large cell lymphoma, is much easier to treat and has a far better prognosis than lung cancer.
The 22-year-old rookie said that his lymphoma was discovered on a visit to his uncle, a physician in Tacoma, when the Red Sox were in Seattle on a West Coast swing.
Lester had been having back pain, which he thought had been exacerbated by a car accident in Boston on Aug. 18. But during the West Coast visit, his uncle detected several enlarged lymph nodes, and ordered additional tests, including an MRI scan.
"I thought I was in the best shape of my life coming in here, pitching every five days and pitching at Fenway Park -- what could be better?" Lester said. "Obviously, there's that denial: why, how could it be me, what did I do wrong?"
Anaplastic large cell lymphoma is classified as a childhood non-Hodgkin's lymphoma, a group of hematologic malignancies with features and a clinical course generally distinct from those that normally affect adults. The disease can occur at any age, but is most common in children and young adults.
Anaplastic large cell lymphoma represents about 2% of all NHLs in adults, but is the second most common T-cell lymphoma. Anaplastic large cell lymphoma represents 10% of childhood lymphomas, and 50% of large cell pediatric lymphomas. The median age of patients is 34, with a male predominance. There is a bimodal age distribution, with peaks in childhood/young adult, and late adulthood.
More than 90% of anaplastic lymphomas arise from T-cells, with the remainder generally displaying a null phenotype, according to the National Cancer Institute.
Patients with anaplastic large cell lymphoma may have a broad range of clinical symptoms, including involvement of lymph nodes and extranodal sites, especially skin and bone. Less frequently, areas of the gastrointestinal tract, lung, pleura, and muscle may be involved, although involvement of the central nervous system or bone marrow is uncommon, according to the NCI.
The disease may manifest in cutaneous or systemic forms. Systemic symptoms such as fever and weight loss are common, and the disease often has a prolonged waxing and waning course.
Anaplastic large cell lymphoma is classified a high-grade lymphoma, which signifies a rapid clinical course, but also bodes well for Lester, as high-grade lymphomas are significantly more responsive to cytotoxic and cytodestructive therapies than low-grade or indolent lymphomas.
Although it is not known what stage lymphoma Lester has, "the outlook for children with localized anaplastic large cell lymphoma is excellent with an expected long-term survival of approximately 90%," according to an NCI-statement.
As with other forms of non-Hodgkin's lymphoma, patients are treated with a short course of combination chemotherapy, combined with central nervous system prophylaxis for only those patients with primary malignancies of the head and neck.
The NCI states that radiation therapy has not been shown to improve survival in patients with anaplastic large cell lymphomas.
Combination chemotherapy regimens commonly used to treat stage I and II disease include:
- COMP: Cytoxan (cyclophosphamide), Oncovin (vincristine), methotrexate, and prednisone
- CHOP: Cytoxan, Adriamycin (doxorubicin), Oncovin, prednisone
- French LMB 89: High dose Cytoxan, Oncovin, prednisone, and Adriamycin, and
- NHL-BFM 90: Alternating courses of chemotherapy containing Decadron (dexamethasone), IFEX (ifosfamide), methotrexate, Ara-C (cytarabine), VePesid (etoposide) with our without Oncovin; and Decadron, Cytoxan, methotrexate, and Adriamycin with or without Oncovin. The number of courses is determined by the extent of resection and by the histology.
For patients with stage III and IV anaplastic large cell lymphoma, those with tumors positive for anaplastic lymphoma kinase, a favorable prognostic marker, have an event-free survival of 70% to 75%.
"Leukemic peripheral blood involvement is uncommon, but when present, appears to be associated with an unfavorable prognosis," the NCI treatment statement says.
Treatment options for stage III and IV include the following regimens:
- APO: Adrimaycin, prednisone, Oncovin
- CHOP vincristine, Adriamycin,, Cytoxan, and prednisone.
- NHL-BFM 90: Oncovin, Adriamycin, prednisone, Cytoxan, Decadron, VePesid, IFEX, Ara-C, methotrexate, Eldisine (vindesine)
Other treatment options being investigated for advance anaplastic large cell lymphoma include single-agent therapy with Velban (vinblastine) in patients with relapse, and the International ALCL 99 regimen, consisting of the NHL-BFM 90 regimen described above, with the addition of Oncovin and methotrexate.