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Classic Hodgkin lymphoma, characterized by multinucleated Hodgkin and Reed-Sternberg cells residing in a mixed infiltrate of nonneoplastic cells, is derived from mature B-cells at the germinal center stage of differentiation. Hodgkin and Reed-Sternberg cells contain monoclonal immunoglobulin gene rearrangements but have lost much of the B-cell specific expression program and have acquired inappropriate gene products. Multiple signaling pathways and transcription factors are deregulated in Hodgkin lymphoma. Although transforming events are incompletely understood, recurrent genetic lesions involve the JAK-STAT and nuclear factor κB pathways. Epstein-Barr virus, which is an important environmental factor in well-described subsets of Hodgkin lymphoma, also leads to nuclear factor κB activation. The inflammatory microenvironment promotes survival and allows escape of Hodgkin and Reed-Sternberg cells from immune attack. Morphologic and immunophenotypic features distinguish the four subtypes of classical Hodgkin lymphoma, accounting for 95 percent of cases, and nodular lymphocyte predominance Hodgkin lymphoma. Hodgkin lymphoma spreads in a predictable, contiguous manner and is classified into four stages, I to IV. Hodgkin lymphoma is treated with the intent to cure the disease in all stages, and long-term survival exceeds 85 percent for all stages. Doxorubicin-containing chemotherapy plays a major role in treatment of all stages of the disease whereas, because of concerns for late toxicities, radiotherapy is used selectively. A valuable diagnostic test for assessment of disease and response to treatment, 18-fluorodeoxyglucose positron emission tomography, is being assessed as a measure of response-adapted treatment. High-dose therapy and autologous transplantation is effective in patients who have relapsed, and several promising new biologic agents are available. Consideration for late treatment effects guides therapy and followup in Hodgkin lymphoma, which disproportionately affects adolescents and young adults. Major treatment challenges include the maintenance of high cure rates with fewer short-term and long-term complications, biomarker identification of the small refractory subgroup, and integration of biologic therapies in the treatment of a frequently curable tumor.

Acronyms and Abbreviations

Acronyms and abbreviations that appear in this chapter include: ABVD, Adriamycin (doxorubicin), bleomycin, vinblastine, dacarbazine; BEACOPP, bleomycin, etoposide, Adriamycin (doxorubicin), cyclophosphamide, vincristine, procarbazine, prednisone; BEAM, bischloroethylnitrosourea (carmustine), etoposide, Ara C (cytarabine), melphalan; CBV, cyclophosphamide, bischloroethylnitrosourea (carmustine), etoposide; COPP, cyclophosphamide, vincristine, procarbazine, prednisone; CT, computed tomography; EBV, Epstein-Barr virus; EBVP, epirubicin, bleomycin, vinblastine, prednisone; EORTC, European Organization for the Research and Treatment of Cancer; FDG, 18-fluorodeoxyglucose; GHSG, German Hodgkin Study Group; HLA, human leukocyte antigen; IL, interleukin; LMP, latent membrane protein; MOPP, mechlorethamine (nitrogen mustard), Oncovin (vincristine), procarbazine, prednisone; MVPP, nitrogen mustard, vinblastine, procarbazine, prednisone; NF-κB, nuclear factor-κB; PET, positron emission tomography; RANKL, receptor activator of nuclear factor κB; STAT, signal transducer and activator of transcription.

Classic Hodgkin lymphoma is a neoplasm of lymphoid tissue, in most cases derived from germinal center B cells, defined by the presence of the malignant Hodgkin and Reed-Sternberg cells with a characteristic immunophenotype and appropriate cellular background. Classic Hodgkin lymphoma accounts for 95 percent of cases and contains four histologic subtypes (nodular sclerosis, ...

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