S-1 Supplement
Reassessing the Standard of Care in Indolent Lymphoma: A Clinical Update to Improve Clinical Practice Mathias Rummel, MD, PhD, Giessen, Germany
Key Words Non-Hodgkin’s lymphoma, NHL, diffuse large B-cell lymphoma, follicular lymphoma, chronic lymphocytic leukemia, small lymphocytic lymphoma, CLL, SLL, Waldenström’s macroglobulinemia Abstract Non-Hodgkin’s lymphoma (NHL) represents a diverse group of hematologic malignancies originating in B or T lymphocytes. Approximately 85% of NHLs are of B-cell origin, with the remainder mostly of T-cell origin. The most common NHL types are diffuse large B-cell lymphoma (31%) and follicular lymphoma (22%). More than 65,000 new cases of NHL develop each year, and approximately 20,000 people with NHL died of the disease 2009. NHL is the seventh most common cancer in the United States, contributes to approximately 4% to 5% of all cancer cases in the United States, and causes approximately 3% of all cancer-related deaths. Currently, nearly 500,000 people are living with the disease or are in remission. Several new and encouraging advances have been made in the treatment of indolent NHL. Although the watch and wait approach still has a role, combined immunochemotherapy remains the standard of care for both first-line and relapsed/refractory disease. As front-line treatment, bendamustine plus rituximab may become a new standard of care, especially for older patients. In contrast, rituximab in combination with chemotherapy followed by rituximab maintenance seems to be the optimal option in patients with relapsed disease. (JNCCN 2010;8[Suppl 6]:S1–S14)
Epidemiology Non-Hodgkin’s lymphoma (NHL) represents a diverse group of hematologic malignancies originating in B or T lymphocytes. Approximately 85% of NHLs are of B-cell origin, with the remainder mostly of T-cell origin.1 The most common NHL types are diffuse large B-cell lymphoma (DLBCL; 31%) and follicular lymphoma (FL; 22%; (Figure 1).2 More than 65,000 new cases of NHL develop each year, and approximately 20,000 people with NHL will
die in 2009.3 NHL is the seventh most common cancer in the United States, contributes to approximately 4% to 5% of all cancer cases in the United States, and causes approximately 3% of all cancer-related deaths.1,4 Currently, nearly 500,000 people are living with the disease or are in remission.4 The age-adjusted incidence of NHL increased more than 75% from 1975 to 2006.4 In addition, the incidence of NHL increases with age. The incidence increases approximately 20-fold between the ages of 20 to 24 years and 60 years, and about 50-fold between the ages of 20 to 24 years and 75 years. The prevalence of histologic subtypes also varies depending on age group.5
Pathophysiology and Classification NHL is classified as indolent, aggressive, or highly aggressive. Indolent lymphoma subtypes include FL, chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL), and marginal zone lymphoma (MZL), which includes mucosa-associated lymphoid tissue (MALT) lymphoma, splenic MZL, and nodal MZL.1 Lymphoplasmacytic lymphoma, also termed Waldenström’s macroglobulinemia (WM), is also considered to be an indolent form of NHL.6 Aggressive NHLs include DLBCL and mantle cell lymphoma (MCL). Highly aggressive NHLs include Burkitt’s lymphoma, lymphoblastic lymphoma, and AIDS-related B-cell lymphoma.6 This clinical update focuses on indolent NHL. The causes of NHL are mostly unknown, and those that are known explain only a small proportion of cases.4 Immunosuppression may be involved, as evidenced by the association of HIV infection with a higher incidence of NHL. In addition, exposure to herbicides, pesticides, and certain viruses such as Epstein-Barr virus
© JNCCN – Journal of the National Comprehensive Cancer Network | Volume 8 Supplement 6 | September 2010