Treatment of non-Hodgkin lymphoma (NHL) depends on the histologic type and stage. Many of the improvements in survival have been made using clinical trials (experimental therapy) that have attempted to improve on the best available accepted therapy (conventional or standard therapy).

Even though standard treatment in patients with lymphomas can cure a significant fraction, numerous clinical trials that explore improvements in treatment are in progress. If possible, patients should be included in these studies. Standardized guidelines for response assessment have been suggested for use in clinical trials.[1]

Late effects of treatment of NHL have been observed. Pelvic radiation therapy and large cumulative doses of cyclophosphamide have been associated with a high risk of permanent sterility.[2] For as many as 2 decades after diagnosis, patients are at a significantly elevated risk for second primary cancers, especially lung, brain, kidney, and bladder cancers and melanoma, Hodgkin lymphoma, and acute nonlymphocytic leukemia.[3-5] 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.[6,7] 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.[4,8-14] Most of these patients show clonal hematopoiesis even before the transplantation, suggesting that the hematologic injury usually occurs during induction or reinduction chemotherapy.[11,15,16] With a median 10-year follow-up after autologous bone marrow transplantation (BMT) with conditioning using cyclophosphamide and total-body radiation therapy, in a series of 605 patients, the incidence of a second malignancy was 21%, and 10% of those were solid tumors.[17] Successful pregnancies with children born free of congenital abnormalities have been reported in young women after autologous BMT.[18]

Aggressive lymphomas are increasingly seen in HIV-positive patients whose treatment requires special consideration. (Refer to the PDQ summary on AIDS-Related Lymphoma Treatment for more information.)

Several unusual presentations of lymphoma occur that often require somewhat modified approaches to staging and therapy. The reader is referred to reviews for a more detailed description of extranodal presentations in the gastrointestinal system,[19-27] thyroid,[28,29] spleen,[30] testis,[31] paranasal sinuses,[32-36] bone,[37,38] orbit,[39-41] and skin.[42-50]

(Refer to the PDQ summary on Primary Central Nervous System Lymphoma Treatment for more information.)

References

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25. Koch P, del Valle F, Berdel WE, et al.: Primary gastrointestinal non-Hodgkin's lymphoma: I. Anatomic and histologic distribution, clinical features, and survival data of 371 patients registered in the German Multicenter Study GIT NHL 01/92. J Clin Oncol 19 (18): 3861-73, 2001.  [PUBMED Abstract]
    
    
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28. Blair TJ, Evans RG, Buskirk SJ, et al.: Radiotherapeutic management of primary thyroid lymphoma. Int J Radiat Oncol Biol Phys 11 (2): 365-70, 1985.  [PUBMED Abstract]
    
    
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40. Uno T, Isobe K, Shikama N, et al.: Radiotherapy for extranodal, marginal zone, B-cell lymphoma of mucosa-associated lymphoid tissue originating in the ocular adnexa: a multiinstitutional, retrospective review of 50 patients. Cancer 98 (4): 865-71, 2003.  [PUBMED Abstract]
    
    
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43. Pandolfino TL, Siegel RS, Kuzel TM, et al.: Primary cutaneous B-cell lymphoma: review and current concepts. J Clin Oncol 18 (10): 2152-68, 2000.  [PUBMED Abstract]
    
    
44. Sarris AH, Braunschweig I, Medeiros LJ, et al.: Primary cutaneous non-Hodgkin's lymphoma of Ann Arbor stage I: preferential cutaneous relapses but high cure rate with doxorubicin-based therapy. J Clin Oncol 19 (2): 398-405, 2001.  [PUBMED Abstract]
    
    
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