Non-Hodgkin's Lymphoma

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SPLENIC MARGINAL ZONE LYMPHOMA

Splenic marginal zone lymphoma is an indolent lymphoma that is marked by massive splenomegaly and peripheral blood and bone marrow involvement, usually without adenopathy.[78,79,80] This type of lymphoma is otherwise known as splenic lymphoma with villous lymphocytes,. Splenectomy may result in prolonged remission.[51,81] Management is similar to that of other low-grade lymphomas and usually involves rituximab alone or rituximab in combination with purine analogs or alkylating agent chemotherapy.[82] Splenic marginal zone lymphoma responds less well to chemotherapy, which would ordinarily be effective for chronic lymphocytic leukemia.[79,80,82] Among small numbers of patients with splenic marginal zone lymphoma (splenic lymphoma with villous lymphocytes) and infection with HCV, the majority attained a complete or partial remission after loss of detectable HCV RNA with treatment using interferon-a with or without ribavirin.[42,83,84][Level of evidence: 3iiiDiii] In contrast, no responses to interferon were seen in six HCV-negative patients.

PRIMARY CUTANEOUS ANAPLASTIC LARGE CELL LYMPHOMA

Primary cutaneous anaplastic large cell lymphoma presents in the skin only with no pre-existing lymphoproliferative disease and no extracutaneous sites of involvement.[85,86] Patients with this type of lymphoma encompass a spectrum ranging from clinically benign lymphomatoid papulosis, marked by localized nodules that may regress spontaneously, to a progressive and systemic disease requiring aggressive doxorubicin-based combination chemotherapy. This spectrum has been called the primary cutaneous CD30-positive T-cell lymphoproliferative disorder. Patients with localized disease usually undergo radiation therapy. With more disseminated involvement, watchful waiting or doxorubicin-based combination chemotherapy is applied.[85,86]

(Refer to the PDQ summaries on Chronic Lymphocytic Leukemia Treatment; Mycosis Fungoides/Sézary Syndrome Treatment; Hairy Cell Leukemia Treatment; and Adult Hodgkin’s Lymphoma Treatment for more information.)

Aggressive NHL

DIFFUSE LARGE CELL LYMPHOMA

Diffuse large B-cell lymphoma is the most common of the non-Hodgkin’s lymphomas and comprises 30% of newly diagnosed cases.[7] Most patients present with rapidly enlarging masses, often with symptoms both locally and systemically (designated B symptoms with fever, recurrent night sweats, or weight loss). The vast majority of patients with localized disease are curable with combined modality therapy or combination chemotherapy alone.[87] For patients with advanced-stage disease, 50% of presenting patients are cured with doxorubicin-based combination chemotherapy and rituximab.[88,89,90]

An International Prognostic Index (IPI) for aggressive NHL (diffuse large cell lymphoma) identifies five significant risk factors prognostic of OS:[91]

1. Age (=60 years of age vs. >60 years of age).
2. Serum lactate dehydrogenase (LDH) (normal vs. elevated).
3. Performance status (0 or 1 vs. 2–4).
4. Stage (stage I or stage II vs. stage III or stage IV).
5. Extranodal site involvement (0 or 1 vs. 2–4).

Patients with two or more risk factors have a less than 50% chance of relapse-free survival and OS at 5 years. This study also identifies patients at high risk of relapse based on specific sites of involvement, including bone marrow, CNS, liver, lung, and spleen. Age-adjusted and stage-adjusted modifications of this IPI are used for younger patients with localized disease.[92] Patients at high risk of relapse may be considered for clinical trials.[93] Molecular profiles of gene expression using DNA microarrays may help to stratify patients in the future for therapies directed at specific targets and to better predict survival after standard chemotherapy.[94,95,96]