Skip to content
My WebMD Sign In, Sign Up

Non-Hodgkin's Lymphoma

Font Size

Cellular Classification



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 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]


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]


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.

Today on WebMD

what is your cancer risk
Integrative Medicine Cancer Quiz
cancer fighting foods
Your Cancer Specialists Doctors You Need To Know

Vitamin D
New Treatments For Non-Hodgkins Lymphoma
Lifestyle Tips for Depression Slideshow
Pets Improve Your Health

WebMD Special Sections