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Non-Hodgkin's Lymphoma

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Cellular Classification


CNS prophylaxis (usually with four to six injections of methotrexate intrathecally) is recommended for patients with paranasal sinus or testicular involvement. Some clinicians are employing high-dose intravenous methotrexate (usually four doses) as an alternative to intrathecal therapy because drug delivery is improved, and patient morbidity is decreased.[97] CNS prophylaxis for bone marrow involvement is controversial; some investigators recommend it, others do not.[98] A retrospective analysis of 605 patients with diffuse large cell lymphoma who did not receive prophylactic intrathecal therapy identified an elevated serum LDH and more than one extranodal site as independent risk factors for CNS recurrence. Patients with both risk factors have a 17% probability of CNS recurrence at 1 year after diagnosis (95% confidence interval [CI], 7%–28%) versus 2.8% (95% CI, 2.7%–2.9%) for the remaining patients.[99][Level of evidence: 3iiiDii] Some cases of large B-cell lymphoma have a prominent background of reactive T-cells and often of histiocytes, so-called T-cell/histocyte-rich large B-cell lymphoma. This subtype of large cell lymphoma has frequent liver, spleen, and bone marrow involvement; however, the outcome is equivalent to that of similarly staged patients with diffuse large B-cell lymphoma.[100,101,102] Some patients with diffuse large B-cell lymphoma at diagnosis have a concomitant indolent small B-cell component; while OS appears similar after multidrug chemotherapy, there is a higher risk of indolent relapses.[103]


Primary mediastinal (thymic) large B-cell lymphoma is a subset of diffuse large cell lymphoma characterized by significant fibrosis on histology.[104,105,106,107,108,109,110] Patients are usually female and young (median age 30–40 years). Patients present with a locally invasive anterior mediastinal mass that may cause respiratory symptoms or superior vena cava syndrome. Therapy and prognosis are the same as for other comparably staged patients with diffuse large cell lymphoma, except for advanced-stage patients with a pleural effusion, who have an extremely poor prognoses (progression-free survival is less than 20%) whether the effusion is cytologically positive or negative. High-dose chemotherapy with hematopoietic stem cell rescue has been applied to these poor prognosis patients. Evidence for this approach is anecdotal.[110]


The natural history of follicular large cell lymphoma remains controversial.[111] While there is agreement about the significant number of long-term disease-free survivors with early stage disease, the curability of patients with advanced disease (stage III or stage IV) remains uncertain. Some groups report a continuous relapse rate similar to the other follicular lymphomas (a pattern of indolent lymphoma).[112] Other investigators report a plateau in freedom-from-progression at levels expected for an aggressive lymphoma (40% at 10 years).[113,114] This discrepancy may be caused by variations in histologic classification between institutions and the rarity of patients with follicular large cell lymphoma. A retrospective review of 252 patients, all treated with anthracycline-containing combination chemotherapy, showed that patients with more than 50% diffuse components on biopsy had a worse OS than other patients with follicular large cell lymphoma.[115] Treatment of these patients is more similar to treatment of aggressive NHL than it is to the treatment of indolent NHL. In support of this approach, treatment with high-dose chemotherapy and autologous hematopoietic peripheral stem cell transplantation shows the same curative potential in patients with follicular large cell lymphoma who relapse as it does in patients with diffuse large cell lymphoma who relapse.[116][Level of evidence: 3iiiA]


WebMD Public Information from the National Cancer Institute

Last Updated: April 02, 2007
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