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INTRODUCTION

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  • Lymphomas are a heterogeneous group of malignancies that arise from malignant transformation of immune cells residing predominantly in lymphoid tissues. Differences in histology have led to the classification of Hodgkin and non-Hodgkin lymphoma (HL and NHL, respectively), which are addressed separately in this chapter.

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HODGKIN LYMPHOMA

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PATHOPHYSIOLOGY

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  • B-cell transcriptional processes are disrupted during malignant transformation, preventing expression of B-cell surface markers and production of immunoglobulin messenger RNA. Alterations in the normal apoptotic pathways favor cell survival and proliferation.

  • Malignant Reed–Sternberg cells overexpress nuclear factor-κ B, which is associated with cell proliferation and antiapoptotic signals. Infections with viral and bacterial pathogens upregulate nuclear factor-κ B. Epstein–Barr virus is found in many, but not all, HL tumors.

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CLINICAL PRESENTATION

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  • Most patients with HL present with a painless, rubbery, enlarged lymph node in the supradiaphragmatic area and commonly have mediastinal nodal involvement. Asymptomatic adenopathy of the inguinal and axillary regions may also be present.

  • Constitutional, or “B,” symptoms (eg, fever, drenching night sweats, and weight loss) are present at diagnosis in approximately 25% of patients with HL.

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DIAGNOSIS AND STAGING

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  • Diagnosis requires the presence of Reed–Sternberg cells in the lymph node biopsy.

  • Staging is performed to provide prognostic information and to guide therapy. Clinical staging is based on noninvasive procedures such as history, physical examination, laboratory tests, and radiography, including positron emission tomography (PET). Pathologic staging is based on biopsy findings of strategic sites (eg, bone marrow, spleen, and abdominal nodes) using an invasive procedure (eg, laparoscopy).

  • At diagnosis, approximately half of patients have localized disease (stages I, II, and IIE) and the others have advanced disease, of which 10% to 15% is stage IV.

  • Prognosis predominantly depends on age and amount of disease; patients older than 65 to 70 years have a lower cure rate than younger patients. Patients with limited stage disease (stages I and II) have a 90% to 95% cure rate, whereas those with advanced disease (stages III and IV) have a 60% to 80% cure rate.

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TREATMENT

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  • Goals of Treatment: The goal is to maximize curability while minimizing short- and long-term treatment-related complications.

  • Treatment options include radiation therapy (RT), chemotherapy, or both (combined-modality therapy). The therapeutic role of surgery is limited, regardless of stage.

  • RT is an integral part of treatment and can be used alone for select patients with early-stage disease, although most patients will receive chemotherapy and radiation.

  • Long-term complications of RT, chemotherapy, and chemoradiotherapy include gonadal dysfunction, secondary malignancies (eg, lung, breast, and GI tract, as well as leukemia), and cardiac disease.

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Initial Chemotherapy
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  • Treat all stages and risk-groups of HL with 8 to 12 weeks of chemotherapy and then obtain a restaging PET-CT (Table 63–1). Most patients with unfavorable disease will require ...

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