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CASE HISTORY • Part 1
A 26-year-old man presents with a lump in his neck that he discovered while shaving. He had noted this several months ago but it regressed and he ignored it until it reappeared several weeks ago. In the past few weeks, he has awakened during the night with drenching sweats and has had an unexplained 5-lb weight loss.
His past medical history and family history are unremarkable. He reports that 2 unrelated friends have been diagnosed with Hodgkin lymphoma in the past 5 years, prompting his concern.
Examination reveals a 3-cm anterior cervical lymph node that is non-tender, rubbery, and freely moveable. The remainder of his examination is normal.
Question
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Hodgkin lymphoma is a distinct type of lymphoma that in many ways offers a paradigm for the diagnosis and treatment of all hematologic malignancies. Although the causes of Hodgkin lymphoma still remain largely unknown, advances in chemo- and radiotherapy have converted this previously lethal disease to one that is highly curable. The first major advance resulted from an understanding of its radiosensitivity and unique mode of anatomic spread, step by step, within the lymphoid system. This in turn led to the successful design of radiation therapy strategies with large fields encompassing not only involved lymph nodes, but also adjacent nodal areas; thus, accurate staging was shown to be of paramount importance. Subsequently, chemotherapy regimens have proven to be curative even in cases of extensive disease. Moreover, combining these 2 treatment modalities has allowed reduction in the toxicity of each. Before these treatment regimens were available, 90% of patients with Hodgkin lymphoma died within 2–5 years, whereas 85%–90% are now curable with modern therapy. Therefore, Hodgkin lymphoma is one of the best examples of a strategic approach to malignancy leading to cure.
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Now that most patients with Hodgkin lymphoma can be cured, the long-term toxicity of the treatments has come into sharp focus, especially long-term vascular/cardiotoxicity and the development of secondary malignancies. Overall, these sequelae have prompted a search for new regimens that are able to combine high efficacy with low toxicity, and to preserve fertility in young adults. The major caveat of this search, of course, is to not reduce curability.
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The incidence of Hodgkin lymphoma (HL) has been linked to several factors, including environmental, social status, infectious agents, and genetic propensity. HL is slightly more common in men and has occurred as clustered cases in families, communities, and schools, suggesting an infectious component. It is also more common in developed countries. Patients who have had infectious mononucleosis or have a positive test for prior Epstein-Barr virus (EBV) infection have a 3-fold increased risk for developing the disease. There is also an increased incidence in individuals with occupational chemical exposures or immune deficiency states. There is also a component of genetic susceptibility; studies of identical twins have suggested a genetic propensity ...