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. 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.)
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. 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. 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:
- Age (=60 years of age vs. >60 years of age).
- Serum lactate
dehydrogenase (LDH) (normal vs. elevated).
- Performance status (0 or 1 vs. 2–4).
- Stage (stage I or stage II vs. stage III or stage IV).
- Extranodal site involvement (0 or 1
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. Patients at high risk of relapse may be considered for clinical
trials. 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]