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EPIDEMIOLOGY

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  • Approximately 72,000 cases of non-Hodgkin lymphoma (NHL) will be diagnosed in 2015 and approximately 20,000 persons will die of the disease in the United States.

  • NHL represents approximately 4.5% of cancers in the United States and 3.5% of cancer deaths, annually.

  • The age-adjusted incidence rates per 100,000 persons for NHL in the United States are 25 for white males, 18 for black males, 17 for white females, and 12 for black females.

  • The risk of NHL in the United States is approximately three times that of several underdeveloped countries and two times that of several comparable industrialized countries.

  • There is a logarithmic increase in annual incidence in both men and women from late teenagers to octogenarians in the United States: males 15 to 19 years of age, 2.3 cases/100,000 persons; males 80 to 84 years of age, 147 cases/100,000 persons; females 15 to 19 years, 1.2 cases/100,000 persons; and females 80 to 84 years, 102 cases per 100,000 persons.

  • Follicular lymphoma represents approximately 25% of NHL cases in the United States but is uncommon in many developing countries and in Asia, especially China and Japan.

  • Diffuse large B-cell lymphoma represents approximately 30% of NHL cases in the United States.

  • The annual incidence of NHL, but not Hodgkin lymphoma, increased significantly between 1972 and 1995 in the United States and western European countries. The increase probably started before 1972 based on European data, but the United States National Cancer Institute did not track specific-site cancer incidence before that date.

  • The rate is still increasing slightly for women and older men in the United States. Orbital adnexal lymphoma and mantle cell lymphoma are increasing at a rate of approximately 5% per year.

  • The evidence that benzene, other solvents, pesticides, herbicides, dyes, various occupations, and other industrial exposures increase the relative risk of lymphoma is disputed and, at this time, is insufficient to reach a level of medical or scientific certainty, according to the International Agency on Research in Cancer.

  • There are instances of familial clustering and an increase in the relative risk of lymphoma in siblings of patients with lymphoma or related hematologic malignancies (eg, myeloma). These so-called nonsyndromic examples of increased familial risk are likely explained by as yet undefined predisposition genes, akin to the Li-Fraumeni syndrome, which is the result of germline inheritance of mutated p53.

  • Several syndromic immunodeficiency states increase the relative risk of lymphoma in family members (see “Immunosuppression and Autoimmunity,” below).

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HISTOLOGIC HETEROGENEITY

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  • The World Health Organization has categorized more than 30 unique histopathologic types of NHL and these are shown in Table 58–1 with their approximate relative frequency. Approximately 88% are B-cell lymphomas and approximately 12% are T-cell lymphomas. There are striking variations in the incidence of various subtypes of NHL in different geographic areas throughout the world.

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Table Graphic Jump Location
TABLE 58–1HISTOLOGIC SUBTYPES AND RELATIVE FREQUENCY OF THE NON-HODGKIN LYMPHOMAS (NHL)

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