Skip to content
My WebMD Sign In, Sign Up

Non-Hodgkin's Lymphoma

Font Size

Cellular Classification

continued...

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]

1|2|3|4|5|6|7|8|9|10|11|12|13

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
HEALTH CHECK
Integrative Medicine Cancer Quiz
QUIZ
 
cancer fighting foods
SLIDESHOW
Your Cancer Specialists Doctors You Need To Know
REFERENCE
 

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

WebMD Special Sections