Changes to This Summary (03 / 06 / 2013)
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.Editorial changes were made to this summary.
Changes to This Summary (02 / 06 / 2013)
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above. Editorial changes were made to this summary.
Treatment Options for Non-Hodgkin Lymphoma
Treatment options for Non-Hodgkin Lymphoma.
Cellular Classification of Childhood NHL
Cellular Classification and Clinical PresentationIn children, non-Hodgkin lymphoma (NHL) is distinct from the more common forms of lymphoma observed in adults. While lymphomas in adults are more commonly low or intermediate grade, almost all NHL that occurs in children is high grade.[1,2,3] The World Health Organization (WHO) has classified NHL on the basis of the following: (1) phenotype (i.e., B-lineage and T-lineage or natural killer [NK] cell lineage) and (2) differentiation (i.e., precursor vs. mature).On the basis of clinical response to treatment, NHL of childhood and adolescence currently falls into the following three therapeutically relevant categories: Mature B-cell NHL (Burkitt and Burkitt-like lymphoma/leukemia and diffuse large B-cell lymphoma).Lymphoblastic lymphoma (primarily precursor T-cell lymphoma and, less frequently, precursor B-cell lymphoma).Anaplastic large cell lymphoma (mature T-cell or null-cell lymphomas).NHL associated with
Stages of Adult Non-Hodgkin Lymphoma
After adult non-Hodgkin lymphoma has been diagnosed, tests are done to find out if cancer cells have spread within the lymph system or to other parts of the body.
Cellular Classification of Adult NHL
A pathologist should be consulted prior to a biopsy because some studies require special preparation of tissue (e.g., frozen tissue). Knowledge of cell surface markers and immunoglobulin and T-cell receptor gene rearrangements may help with diagnostic and therapeutic decisions. The clonal excess of light-chain immunoglobulin may differentiate malignant from reactive cells. Since the prognosis and the approach to treatment are influenced by histopathology, outside biopsy specimens should be carefully reviewed by a hematopathologist who is experienced in diagnosing lymphomas. Although lymph node biopsies are recommended whenever possible, sometimes immunophenotypic data are sufficient to allow diagnosis of lymphoma when fine-needle aspiration cytology is preferred.[1,2]Historical Classification SystemsHistorically, uniform treatment of patients with non-Hodgkin lymphoma (NHL) has been hampered by the lack of a uniform classification system. In 1982, results of a consensus
Late Effects of Treatment for Adult NHL
Late effects of treatment for non-Hodgkin lymphoma (NHL) have been observed. Pelvic radiation therapy and large cumulative doses of cyclophosphamide have been associated with a high risk of permanent sterility. For as many as three decades after diagnosis, patients are at a significantly elevated risk for second primary cancers, especially the following:[1,2,3]Lung cancer.Brain cancer.Kidney cancer.Bladder cancer.Melanoma.Hodgkin lymphoma.Acute nonlymphocytic leukemia.Left ventricular dysfunction was a significant late effect in long-term survivors of high-grade NHL who received more than 200 mg/m² of doxorubicin.[4,5]Myelodysplastic syndrome and acute myelogenous leukemia are late complications of myeloablative therapy with autologous bone marrow or peripheral blood stem cell support, as well as conventional chemotherapy-containing alkylating agents.[1,6,7,8,9,10,11,12,13] Most of these patients show clonal hematopoiesis even before the transplantation, suggesting that the
Treatment for Aggressive, Stage I and Contiguous Stage II Adult NHL
Patients with stage I or contiguous stage II diffuse large B-cell lymphoma are candidates for combination chemotherapy with or without involved-field radiation therapy (IF-XRT).The following drug combinations are referred to in this section:R-CHOP: rituximab, an anti-CD20 monoclonal antibody, + cyclophosphamide + doxorubicin + vincristine + prednisone.Standard Treatment Options for Aggressive, Stage I and Contiguous Stage II Adult NHLStandard treatment options for aggressive, stage I and contiguous stage II adult NHL include the following:R-CHOP with or without IF-XRT.R-CHOP with or without IF-XRTFour prospective randomized trials have evaluated the comparison of CHOP or more intensive CHOP-based chemotherapy alone versus combined–modality therapy with CHOP and IF-XRT.[1,2,3,4,5]Evidence (CHOP vs. CHOP with IF-XRT): In a randomized trial with 7 years' median follow-up, 576 patients older than 60 years with early-stage disease received four cycles of CHOP with or without IF-XRT;
Aggressive non-Hodgkin lymphoma (NHL) includes the following subtypes:Diffuse large B-cell lymphoma.Mediastinal large B-cell lymphoma (primary mediastinal large B-cell lymphoma).Follicular large cell lymphoma.Anaplastic large cell lymphoma.Extranodal NK-/T-cell lymphoma.Lymphomatoid granulomatosis.Angioimmunoblastic T-cell lymphoma.Peripheral T-cell lymphoma.Enteropathy-type intestinal T-cell lymphoma.Intravascular large B-cell lymphoma (intravascular lymphomatosis).Burkitt lymphoma/diffuse small noncleaved-cell lymphoma.Lymphoblastic lymphoma.Adult T-cell leukemia/lymphoma.Mantle cell lymphoma.Polymorphic posttransplantation lymphoproliferative disorder.True histiocytic lymphoma.Primary effusion lymphoma.Diffuse Large B-cell LymphomaDiffuse large B-cell lymphoma (DLBCL) is the most common of the NHLs and comprises 30% of newly diagnosed cases. Most patients present with rapidly enlarging masses, often with both local and systemic symptoms
Low-Stage Childhood NHL Treatment
Patients with stage I and II disease have an excellent prognosis, regardless of histology. A Children's Cancer Group study demonstrated that pulsed chemotherapy with cyclophosphamide, vincristine, methotrexate, and prednisone (COMP) administered for 6 months for low-stage (stage I or II) nonlymphoblastic non-Hodgkin lymphoma (NHL) was equivalent to 18 months of therapy with radiation to sites of disease, resulting in more than 85% disease-free survival (DFS) and more than 90% overall survival (OS). However, patients with lymphoblastic lymphoma had a much inferior outcome.[1,2] A Pediatric Oncology Group (POG) study tested 9 weeks of short, pulsed chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP), with or without radiation to involved sites and with or without 24 weeks of maintenance chemotherapy. The results showed no benefit of radiation or maintenance chemotherapy, but the DFS for nonlymphoblastic lymphoma was superior to that of