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Hodgkin lymphoma is a hematopoietic malignancy with unique epidemiologic features that vary depending on the geographic region of the patient. Genetic and environmental contributions to the pathogenesis of the disease are not completely understood and represent an active area of research. Progression of Hodgkin lymphoma initially occurs along functionally contiguous lymph nodes. If untreated, extranodal involvement eventually results from hematogenous dissemination of neoplastic cells to the liver, lungs, bones, bone marrow, and other tissues. The most common clinical presentation is one of painless lymphadenopathy previously attributed to infectious or inflammatory etiologies. Other signs and symptoms vary based on the involved nodal sites and their compression of adjacent organs and tissues. Cytokine production by malignant cells results in constitutional symptoms including anorexia, pruritus, weight loss, night sweats, and fever in some patients.

Providing care for children with Hodgkin lymphoma challenges the pediatric and radiation oncology team to accomplish the tandem goals of optimizing disease control and limiting long-term morbidity.1 Historically, treatment approaches mirrored those used for adults, but outcome was compromised by unacceptable musculoskeletal hypoplasia resulting from high dosage radiation of large volumes in physically immature children. Pediatric patients are also more likely to experience cardiovascular toxicity associated with anthracycline chemotherapy and thoracic radiation, which may predispose them to early mortality associated with cardiomyopathy and coronary artery disease.2 There are also specific gender-related predispositions to treatment effects. Boys are more vulnerable to gonadal toxicity following cumulative doses of alkylating agent chemotherapy used in primary treatment regimens, whereas girls generally maintain ovarian function unless chemotherapy is combined with infradiaphragmatic radiation. Conversely, girls treated with anthracycline chemotherapy, particularly if younger than age 5 years, are more likely than boys to experience cardiotoxicity. Likewise, young women treated with thoracic radiation during puberty have a substantially increased risk of a breast malignancy that is not observed in their male counterparts.3 Because of these unique developmental and gender-related predispositions to therapy effects, there is no single treatment method that is ideal for all patients. Contemporary treatment uses a risk-adapted approach that considers the prognostic features of the disease presentation in the context of a patient’s predisposition to long-term toxicity related to age and gender.

Etiology and Epidemiology

The epidemiologic, clinical, and histologic features of Hodgkin lymphoma are different in economically developed and developing countries.4 Epidemiologic studies support three distinct forms of Hodgkin lymphoma attributable to interactions of environmental and host factors.5 The childhood form (age 14 years or younger) is characterized by a high incidence of the mixed cellularity histologic subtype in children living in poorer socioeconomic environments. The young adult form (ages 15 to 34 years) shows a predominance of the nodular sclerosing histologic subtype in Caucasian adolescents and young adults in developed countries. An older adult form (ages 55 to 74 years) produces the characteristic bimodal distribution of incidence of the disease in industrialized countries. In developing countries, the early peak occurs before adolescence.


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