Childhood Non-Hodgkin Lymphoma Treatment (Professional) (cont.)
IN THIS ARTICLE
Lymphoproliferative Disease Associated With Immunodeficiency in Children
Regardless of the etiology of the immune defect, immunodeficient children with lymphoma have a worse prognosis than does the general population with non-Hodgkin lymphoma (NHL).[1,2,3,4] One potential exception is the more indolent low-grade lymphomas (e.g., mucosa-associated lymphoid tissue [MALT] lymphomas), which have developed in patients with common variable immunodeficiency or other immunodeficient states.[5,6] If the disease is localized and amenable to complete surgical resection and/or radiation therapy, the outcome is quite favorable; however, most NHL in this population is high-stage (stage III or IV) and requires systemic cytotoxic therapy. These patients usually tolerate cytotoxic therapy poorly, with increased morbidity and mortality due to increased infectious complications and often increased end-organ toxicities. (Refer to the PDQ summary on Adult Non-Hodgkin Lymphoma Treatment for more information about MALT lymphomas.)
In the era of highly active antiretroviral therapy, children with human immunodeficiency virus and NHL should be treated with standard chemotherapy regimens for NHL, but careful attention to prophylaxis against and early detection of infection is warranted.[1,7] Patients with primary immunodeficiency can achieve complete and durable remissions with standard chemotherapy regimens for NHL, though again, toxicity is increased. Recurrences in these patients are common and may not represent the same clonal disease. Immunologic correction through allogeneic stem cell transplantation is often required to prevent recurrences. Patients with DNA repair defects (e.g., ataxia-telangiectasia) are particularly difficult to treat.[4,9] Cytotoxic agents produce much more toxicity and greatly increase the risk of secondary malignancies in these patients. Survival is rare at 5 years postdiagnosis.
In posttransplant lymphoproliferative disease (PTLD), first-line therapy is the reduction of immunosuppression, as can be tolerated.[3,10] Rituximab, an anti-CD20 antibody, has been used with some success, but data for its use in children are sparse. Rituximab plus low-intensity chemotherapy may also be effective, even in PTLDs with the t(8;14) Burkitt lymphoma marker.[Level of evidence: 3iiDiii] Patients with T-cell or Hodgkin-like PTLD are usually treated with standard lymphoma-specific chemotherapy regimens.[12,13,14,15]
Standard Treatment Options
Treatment Options Under Clinical Evaluation
The following is an example of a national or international clinical trial that is currently being conducted. Information about ongoing clinical trials is available from the NCI Web site.
eMedicineHealth Public Information from the National Cancer Institute
This information is produced and provided by the National Cancer Institute (NCI). The information in this topic may have changed since it was written. For the most current information, contact the National Cancer Institute via the Internet web site at http://cancer.gov or call 1-800-4-CANCER
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