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DEFINITION

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  • The neoplasm of lymphoid tissue in most cases is derived from germinal center B cells, defined by the presence of the Reed-Sternberg cells or its mononuclear variant Hodgkin cells with a characteristic immunophenotype and appropriate cellular background.

  • The Reed-Sternberg and Hodgkin cell, the neoplastic cells defining Hodgkin disease, are considered of B-cell origin based on their clonal immunoglobulin gene rearrangements.

  • Classic Hodgkin disease accounts for 95% of cases and contains four histologic subtypes that are distinguished on the basis of microscopic appearance and relative proportions of Reed-Sternberg cells, lymphocytes, and fibrosis: nodular sclerosis, mixed cellularity, lymphocyte-depleted, or lymphocyte-rich Hodgkin disease. A fifth subtype, nodular lymphocyte predominance has been added to the four classic histologic types (Table 59–1).

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TABLE 59–1CLASSIFICATION OF HODGKIN LYMPHOMA
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EPIDEMIOLOGY

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  • In 2014 in the United Stated, there were 9190 cases of Hodgkin lymphoma.

  • Incidence rate is influenced by socioeconomic and environmental factors.

  • There is a bimodal age distribution, with a peak between ages 15 to 34 and in those older than age 60 years (Figure 59–1).

  • Nodular sclerosis subtype predominates in young adults.

  • Mixed cellularity subtype predominates in older ages.

  • Presence of the Epstein-Barr virus (EBV) in Reed-Sternberg and Hodgkin cells is more common in less-developed countries and in pediatric and older adult cases.

  • Role for EBV in etiology is suggested by evidence that serologically confirmed mononucleosis confers a threefold risk for Hodgkin disease in young adults.

  • Increased risk among siblings and close relatives suggests genetic factors may contribute to disease susceptibility.

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FIGURE 59–1

The graph depicts the incidence of Hodgkin lymphoma as a function of age among American males and females, 2000 to 2011. (Data from the Surveillance, Epidemiology, and End Results (SEER) Program (www.seer.cancer.gov) Research Data (1973–2011), National Cancer Institute, DCCPS, Surveillance Research Program, Surveillance Systems Branch, released April 2014, based on the November 2013 submission; 2014. Source: Williams Hematology, 9th ed, Chap. 97, Fig. 97–1.)

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ETIOLOGY AND PATHOGENESIS

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  • Reed-Sternberg cells are relatively large cells that typically have bilobed nuclei with prominent eosinophilic nucleoli separated by a clear space from a thickened nuclear membrane (Figure 59–2).

  • Reed-Sternberg cells express CD30 in virtually all and CD15 in the majority of cases of classic Hodgkin lymphoma (Figure 59–3).

  • Reed-Sternberg cells represent monoclonal outgrowths of germinal center B cells that have incurred extensive somatic mutations, most likely in the course of the immune response to antigen.

  • Mononuclear Reed-Sternberg cell variants, referred to as Hodgkin cells, have similar nuclear ...

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