Sections View Full Chapter Figures Tables Videos Full Chapter Figures Tables Videos Supplementary Content ++ DEFINITION ++ Neoplasm of lymphoid tissue in most cases 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 its clonal immunoglobulin gene rearrangements. Classic Hodgkin disease accounts for 95 percent 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 (see Table 59–1). ++Table Graphic Jump LocationTABLE 59–1CLASSIFICATION OF HODGKIN LYMPHOMAView Table|Favorite Table|Download (.pdf) TABLE 59–1 CLASSIFICATION OF HODGKIN LYMPHOMA Histologic Subtype Immunophenotype Nodular lymphocyte-predominant CD20+ CD30– CD15– Ig+ Classic CD20 –*CD30+ CD15+ Ig– Nodular sclerosis Mixed cellularity Lymphocyte-rich Lymphocyte-depleted *Infrequently positive.Source: Williams Hematology, 8th ed, Chap. 99, Table 99–1, p. 1528. ++ EPIDEMIOLOGY ++ In 2009 in the United Stated, there were 8510 cases of Hodgkin lymphoma. Incidence rate is influenced by socioeconomic and environmental factors. Bimodal age distribution: peak between ages 15 to 34 and in those older than age 60 years. Nodular sclerosis predominates in young adults. Mixed cellularity 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. ++ ETIOLOGY AND PATHOGENESIS ++ 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 (see Fig. 59–1). 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 characteristics and may represent Reed-Sternberg cells cut in a plane that shows only one lobe of the nucleus. Nearly all Hodgkin and Reed-Sternberg cells have rearranged and somatically mutated immunoglobulin VH genes (IgHV). It is possible that Hodgkin and Reed-Sternberg cells originate from a preapoptotic germinal center B cell with unfavorable mutations that has escaped negative selection. Karyotypes are usually hyperdiploid with structural abnormalities but without pathognomonic chromosomal aberrations. Reed-Sternberg cells secrete a variety of cytokines and chemokines that may be responsible for the recruitment of nonmalignant cells that comprise the bulk of the cells in the tumor population. Hodgkin and Reed-Sternberg cells show a global loss of their B-cell phenotype, retaining only B-cell features associated ... GET ACCESS TO THIS RESOURCE Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth Get Free Access Through Your Institution Contact your institution's library to ask if they subscribe to McGraw-Hill Medical Products. What is MyAccess? Create a FREE MyAccess profile to: Use this site remotely Bookmark your favorite content Track your self-assessment progress and more!