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Non-Hodgkin's Lymphoma

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Cellular Classification

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ADULT T-CELL LEUKEMIA/LYMPHOMA

Adult T-cell leukemia/lymphoma is caused by infection with the retrovirus human T-cell lymphotropic virus type I and is frequently associated with lymphadenopathy, hypercalcemia, circulating leukemic cells, bone and skin involvement, hepatosplenomegaly, a rapidly progressive course, and poor response to chemotherapy.[183,184] The combination of zidovudine and interferon-a has activity against adult T-cell leukemia/lymphoma, even for patients who failed previous cytotoxic therapy. Durable remissions are seen in 66% of presenting patients with this combination, but long-term disease-free survival rates are not yet available.[185,186,187]

MANTLE CELL LYMPHOMA

Mantle cell lymphoma is found in lymph nodes, the spleen, bone marrow, blood, and sometimes the gastrointestinal system (lymphomatous polyposis).[4,188,189] Mantle cell lymphoma is characterized by CD5-positive follicular mantle B cells, a translocation of chromosomes 11 and 14, and an overexpression of the cyclin D1 protein.[190] Like the low-grade lymphomas, mantle cell lymphoma appears incurable with anthracycline-based chemotherapy and occurs in older patients with generally asymptomatic advanced-stage disease.[191] The median survival, however, is significantly shorter (3–5 years) than that of other lymphomas, and this histology is now considered to be an aggressive lymphoma.[192] A diffuse pattern and the blastoid variant have an aggressive course with shorter survival, while the mantle zone type may have a more indolent course.[43,193] A high cell proliferation rate (increased Ki-67, mitotic index, beta-2-microglobulin) may be associated with a poorer prognosis.[190,194] It is unclear which chemotherapeutic approach offers the best long-term survival in this clinicopathologic entity; refractoriness to chemotherapy is a usual feature.[192,195,196,197,198,199,200] Many investigators are exploring high-dose therapy with stem cell/marrow support or the use of interferon or anti-CD20 antibodies after CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) chemotherapy.[197,198,199,201,202,203,204,205,206,207,208] Thus far, randomized trials have not shown OS benefits from these newer approaches.[208] Bortezomib shows response rates close to 50% in relapsed patients, prompting clinical trials combining this proteasome inhibitor with rituximab and cytotoxic agents in first-line therapy.[209,210,211][Level of evidence: 3iiiDiii]

POLYMORPHIC POSTTRANSPLANTATION LYMPHOPROLIFERATIVE DISORDER (PTLD)

Patients who undergo transplantation of the heart, lung, liver, kidney, or pancreas usually require life-long immunosuppression. This may result in PTLD in 1% to 3% of recipients, which appears as an aggressive lymphoma.[212] Pathologists can distinguish a polyclonal B-cell hyperplasia from a monoclonal B-cell lymphoma; both are almost always associated with EBV.[213] Poor performance status, grafted organ involvement, high IPI, elevated LDH, and multiple sites of disease are poor prognostic factors for PTLD.[214,215] In some cases, withdrawal of immunosuppression results in eradication of the lymphoma.[216] When this is unsuccessful or not feasible, a trial of rituximab may be considered, because it has shown durable remissions in approximately 60% of patients and a favorable toxicity profile.[217] Sometimes, a combination of acyclovir and interferon-a has been used.[212,218] If these measures fail, doxorubicin-based combination chemotherapy is recommended, though most patients can avoid cytotoxic therapy.[219] Localized presentations can be controlled with surgery or radiation therapy alone. These localized mass lesions, which may grow over a period of months, are often phenotypically polyclonal and tend to occur within weeks or a few months after transplantation.[213] Multifocal, rapidly progressive disease occurs late after transplantation (>1 year) and is usually phenotypically monoclonal and associated with EBV.[220] These patients may have durable remissions using standard chemotherapy regimens for aggressive lymphoma.[220,221,222] Instances of EBV-negative PTLD occur late (median, 5 years posttransplant) and have particularly poor prognoses.[223] A sustained clinical response after failure from chemotherapy was attained using an immunotoxin (anti-CD22 B-cell surface antigen antibody linked with ricin, a plant toxin).[224] An anti-interleukin-6 monoclonal antibody is also under clinical evaluation.[225]

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WebMD Public Information from the National Cancer Institute

Last Updated: April 02, 2007
This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.

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